Product References
Myopathy With SQSTM1 and TIA1 Variants: Clinical and Pathological Features.
Frontiers in neurology
Niu Z,Pontifex CS,Berini S,Hamilton LE,Naddaf E,Wieben E,Aleff RA,Martens K,Gruber A,Engel AG,Pfeffer G,Milone M
Sat Apr 09 00:00:00 EDT 2022
Association between diabetes and causes of dementia: Evidence from a clinicopathological study.
Dementia & neuropsychologia
Matioli MNPDS,Suemoto CK,Rodriguez RD,Farias DS,da Silva MM,Leite REP,Ferretti-Rebustini REL,Pasqualucci CA,Jacob W,Grinberg LT,Nitrini R
Sat Apr 09 00:00:00 EDT 2022
Properties of LINE-1 proteins and repeat element expression in the context of amyotrophic lateral sclerosis.
Mobile DNA
Pereira GC,Sanchez L,Schaughency PM,Rubio-Roldán A,Choi JA,Planet E,Batra R,Turelli P,Trono D,Ostrow LW,Ravits J,Kazazian HH,Wheelan SJ,Heras SR,Mayer J,García-Pérez JL,Goodier JL
Fri Apr 08 00:00:00 EDT 2022
Cytoplasmic Relocalization of TAR DNA-Binding Protein 43 Is Not Sufficient to Reproduce Cellular Pathologies Associated with ALS In vitro.
Frontiers in molecular neuroscience
Wobst HJ,Wesolowski SS,Chadchankar J,Delsing L,Jacobsen S,Mukherjee J,Deeb TZ,Dunlop J,Brandon NJ,Moss SJ
Thu Apr 15 00:00:00 EDT 2021
Cytoplasmic Relocalization of TAR DNA-Binding Protein 43 Is Not Sufficient to Reproduce Cellular Pathologies Associated with ALS In vitro.
Frontiers in molecular neuroscience
Wobst HJ,Wesolowski SS,Chadchankar J,Delsing L,Jacobsen S,Mukherjee J,Deeb TZ,Dunlop J,Brandon NJ,Moss SJ
Thu Apr 15 00:00:00 EDT 2021
DGCR8 Promotes Neural Progenitor Expansion and Represses Neurogenesis in the Mouse Embryonic Neocortex.
Frontiers in neuroscience
Hoffmann N,Weise SC,Marinaro F,Vogel T,De Pietri Tonelli D
Thu Oct 01 00:00:00 EDT 2020
Decreased Levels of Foldase and Chaperone Proteins Are Associated with an Early-Onset Amyotrophic Lateral Sclerosis.
Frontiers in molecular neuroscience
Filareti M,Luotti S,Pasetto L,Pignataro M,Paolella K,Messina P,Pupillo E,Filosto M,Lunetta C,Mandrioli J,Fuda G,Calvo A,Chiò A,Corbo M,Bendotti C,Beghi E,Bonetto V
Thu Oct 01 00:00:00 EDT 2020
Neuronal Expression of UBQLN2P497H Exacerbates TDP-43 Pathology in TDP-43G348C Mice through Interaction with Ubiquitin.
Molecular neurobiology
Picher-Martel V,Renaud L,Bareil C,Julien JP
Mon Jul 01 00:00:00 EDT 2019
Neuronal Expression of UBQLN2P497H Exacerbates TDP-43 Pathology in TDP-43G348C Mice through Interaction with Ubiquitin.
Molecular neurobiology
Picher-Martel V,Renaud L,Bareil C,Julien JP
Mon Jul 01 00:00:00 EDT 2019
Biomarkers for diseases with TDP-43 pathology.
Molecular and cellular neurosciences
Steinacker P,Barschke P,Otto M
Sat Jun 01 00:00:00 EDT 2019
Memory Decline and Its Reversal in Aging and Neurodegeneration Involve miR-183/96/182 Biogenesis.
Molecular neurobiology
Jawaid A,Woldemichael BT,Kremer EA,Laferriere F,Gaur N,Afroz T,Polymenidou M,Mansuy IM
Wed May 01 00:00:00 EDT 2019
Recapitulation of Pathological TDP-43 Features in Immortalized Lymphocytes from Sporadic ALS Patients.
Molecular neurobiology
Posa D,Martínez-González L,Bartolomé F,Nagaraj S,Porras G,Martínez A,Martín-Requero Á
Mon Apr 01 00:00:00 EDT 2019
Motor neuron degeneration, severe myopathy and TDP-43 increase in a transgenic pig model of SOD1-linked familiar ALS.
Neurobiology of disease
Crociara P,Chieppa MN,Vallino Costassa E,Berrone E,Gallo M,Lo Faro M,Pintore MD,Iulini B,D'Angelo A,Perona G,Botter A,Formicola D,Rainoldi A,Paulis M,Vezzoni P,Meli F,Peverali FA,Bendotti C,Trolese MC,Pasetto L,Bonetto V,Lazzari G,Duchi R,Perota A,Lagutina I,Quadalti C,Gennero MS,Dezzutto D,Desiato R,Boido M,Ghibaudi M,Valentini MC,Caramelli M,Galli C,Casalone C,Corona C
Mon Apr 01 00:00:00 EDT 2019
Aggregation of the nucleic acid-binding protein TDP-43 occurs via distinct routes that are coordinated with stress granule formation.
The Journal of biological chemistry
Chen Y,Cohen TJ
Fri Mar 08 00:00:00 EST 2019
Aggregation of the nucleic acid-binding protein TDP-43 occurs via distinct routes that are coordinated with stress granule formation.
The Journal of biological chemistry
Chen Y,Cohen TJ
Fri Mar 08 00:00:00 EST 2019
Structure of Calcarisporiella thermophila Hsp104 Disaggregase that Antagonizes Diverse Proteotoxic Misfolding Events.
Structure (London, England : 1993)
Michalska K,Zhang K,March ZM,Hatzos-Skintges C,Pintilie G,Bigelow L,Castellano LM,Miles LJ,Jackrel ME,Chuang E,Jedrzejczak R,Shorter J,Chiu W,Joachimiak A
Tue Mar 05 00:00:00 EST 2019
Methods to Investigate the Molecular Basis of Progranulin Actions on Brain and Behavior In Vivo Using Knockout Mice.
Methods in molecular biology (Clifton, N.J.)
Boddaert J,Wils H,Kumar-Singh S
Mon Mar 04 00:00:00 EST 2019
Methods to Investigate the Molecular Basis of Progranulin Actions on Brain and Behavior In Vivo Using Knockout Mice.
Methods in molecular biology (Clifton, N.J.)
Boddaert J,Wils H,Kumar-Singh S
Mon Mar 04 00:00:00 EST 2019
TDP-43 levels are higher in platelets from patients with sporadic amyotrophic lateral sclerosis than in healthy controls.
Neurochemistry international
Hishizawa M,Yamashita H,Akizuki M,Urushitani M,Takahashi R
Fri Mar 01 00:00:00 EST 2019
Pathological Modification of TDP-43 in Amyotrophic Lateral Sclerosis with SOD1 Mutations.
Molecular neurobiology
Jeon GS,Shim YM,Lee DY,Kim JS,Kang M,Ahn SH,Shin JY,Geum D,Hong YH,Sung JJ
Fri Mar 01 00:00:00 EST 2019
Pathological Modification of TDP-43 in Amyotrophic Lateral Sclerosis with SOD1 Mutations.
Molecular neurobiology
Jeon GS,Shim YM,Lee DY,Kim JS,Kang M,Ahn SH,Shin JY,Geum D,Hong YH,Sung JJ
Fri Mar 01 00:00:00 EST 2019
Physiologically Important Electrolytes as Regulators of TDP-43 Aggregation and Droplet-Phase Behavior.
Biochemistry
Sun Y,Medina Cruz A,Hadley KC,Galant NJ,Law R,Vernon RM,Morris VK,Robertson J,Chakrabartty A
Tue Feb 12 00:00:00 EST 2019
Rasagiline for amyotrophic lateral sclerosis: A randomized, controlled trial.
Muscle & nerve
Statland JM,Moore D,Wang Y,Walsh M,Mozaffar T,Elman L,Nations SP,Mitsumoto H,Fernandes JA,Saperstein D,Hayat G,Herbelin L,Karam C,Katz J,Wilkins HM,Agbas A,Swerdlow RH,Santella RM,Dimachkie MM,Barohn RJ
Fri Feb 01 00:00:00 EST 2019
ALS-implicated protein TDP-43 sustains levels of STMN2, a mediator of motor neuron growth and repair.
Nature neuroscience
Klim JR,Williams LA,Limone F,Guerra San Juan I,Davis-Dusenbery BN,Mordes DA,Burberry A,Steinbaugh MJ,Gamage KK,Kirchner R,Moccia R,Cassel SH,Chen K,Wainger BJ,Woolf CJ,Eggan K
Fri Feb 01 00:00:00 EST 2019
ALS-implicated protein TDP-43 sustains levels of STMN2, a mediator of motor neuron growth and repair.
Nature neuroscience
Klim JR,Williams LA,Limone F,Guerra San Juan I,Davis-Dusenbery BN,Mordes DA,Burberry A,Steinbaugh MJ,Gamage KK,Kirchner R,Moccia R,Cassel SH,Chen K,Wainger BJ,Woolf CJ,Eggan K
Fri Feb 01 00:00:00 EST 2019
Premature polyadenylation-mediated loss of stathmin-2 is a hallmark of TDP-43-dependent neurodegeneration.
Nature neuroscience
Melamed Z,López-Erauskin J,Baughn MW,Zhang O,Drenner K,Sun Y,Freyermuth F,McMahon MA,Beccari MS,Artates JW,Ohkubo T,Rodriguez M,Lin N,Wu D,Bennett CF,Rigo F,Da Cruz S,Ravits J,Lagier-Tourenne C,Cleveland DW
Fri Feb 01 00:00:00 EST 2019
Premature polyadenylation-mediated loss of stathmin-2 is a hallmark of TDP-43-dependent neurodegeneration.
Nature neuroscience
Melamed Z,López-Erauskin J,Baughn MW,Zhang O,Drenner K,Sun Y,Freyermuth F,McMahon MA,Beccari MS,Artates JW,Ohkubo T,Rodriguez M,Lin N,Wu D,Bennett CF,Rigo F,Da Cruz S,Ravits J,Lagier-Tourenne C,Cleveland DW
Fri Feb 01 00:00:00 EST 2019
Brain banking in low and middle-income countries: Raison D'être for the Ibadan Brain Ageing, Dementia And Neurodegeneration (IBADAN) Brain Bank Project.
Brain research bulletin
Akinyemi RO,Salami A,Akinyemi J,Ojagbemi A,Olopade F,Coker M,Farombi T,Nweke M,Arulogun O,Jegede A,Owolabi M,Kalaria RN,Ogunniyi A
Fri Feb 01 00:00:00 EST 2019
Mutant TDP-43 Causes Early-Stage Dose-Dependent Motor Neuron Degeneration in a TARDBP Knockin Mouse Model of ALS.
Cell reports
Ebstein SY,Yagudayeva I,Shneider NA
Tue Jan 08 00:00:00 EST 2019
Mutant TDP-43 Causes Early-Stage Dose-Dependent Motor Neuron Degeneration in a TARDBP Knockin Mouse Model of ALS.
Cell reports
Ebstein SY,Yagudayeva I,Shneider NA
Tue Jan 08 00:00:00 EST 2019
Single-copy expression of an amyotrophic lateral sclerosis-linked TDP-43 mutation (M337V) in BAC transgenic mice leads to altered stress granule dynamics and progressive motor dysfunction.
Neurobiology of disease
Gordon D,Dafinca R,Scaber J,Alegre-Abarrategui J,Farrimond L,Scott C,Biggs D,Kent L,Oliver PL,Davies B,Ansorge O,Wade-Martins R,Talbot K
Tue Jan 01 00:00:00 EST 2019
Single-copy expression of an amyotrophic lateral sclerosis-linked TDP-43 mutation (M337V) in BAC transgenic mice leads to altered stress granule dynamics and progressive motor dysfunction.
Neurobiology of disease
Gordon D,Dafinca R,Scaber J,Alegre-Abarrategui J,Farrimond L,Scott C,Biggs D,Kent L,Oliver PL,Davies B,Ansorge O,Wade-Martins R,Talbot K
Tue Jan 01 00:00:00 EST 2019
Neurons selectively targeted in frontotemporal dementia reveal early stage TDP-43 pathobiology.
Acta neuropathologica
Nana AL,Sidhu M,Gaus SE,Hwang JL,Li L,Park Y,Kim EJ,Pasquini L,Allen IE,Rankin KP,Toller G,Kramer JH,Geschwind DH,Coppola G,Huang EJ,Grinberg LT,Miller BL,Seeley WW
Tue Jan 01 00:00:00 EST 2019
Impaired Cu-Zn Superoxide Dismutase (SOD1) and Calcineurin (Cn) Interaction in ALS: A Presumed Consequence for TDP-43 and Zinc Aggregation in Tg SOD1G93A Rodent Spinal Cord Tissue.
Neurochemical research
Kim JM,Billington E,Reyes A,Notarianni T,Sage J,Agbas E,Taylor M,Monast I,Stanford JA,Agbas A
Tue Jan 01 00:00:00 EST 2019
Development of an automated capillary nano-immunoassay-Simple Western assay-to quantify total TDP43 protein in human platelet samples.
Analytical and bioanalytical chemistry
Fourier A,Escal J,Bernard E,Lachman I,Perret-Liaudet A,Leblanc P,Quadrio I
Tue Jan 01 00:00:00 EST 2019
TDP-43 extracted from frontotemporal lobar degeneration subject brains displays distinct aggregate assemblies and neurotoxic effects reflecting disease progression rates.
Nature neuroscience
Laferrière F,Maniecka Z,Pérez-Berlanga M,Hruska-Plochan M,Gilhespy L,Hock EM,Wagner U,Afroz T,Boersema PJ,Barmettler G,Foti SC,Asi YT,Isaacs AM,Al-Amoudi A,Lewis A,Stahlberg H,Ravits J,De Giorgi F,Ichas F,Bezard E,Picotti P,Lashley T,Polymenidou M
Tue Jan 01 00:00:00 EST 2019
Impaired Nucleoporins Are Present in Sporadic Amyotrophic Lateral Sclerosis Motor Neurons that Exhibit Mislocalization of the 43-kDa TAR DNA-Binding Protein.
Journal of clinical neurology (Seoul, Korea)
Aizawa H,Yamashita T,Kato H,Kimura T,Kwak S
Tue Jan 01 00:00:00 EST 2019
Transcription-Dependent Formation of Nuclear Granules Containing FUS and RNA Pol II.
Biochemistry
Thompson VF,Victor RA,Morera AA,Moinpour M,Liu MN,Kisiel CC,Pickrel K,Springhower CE,Schwartz JC
Wed Dec 26 00:00:00 EST 2018
Dichotomous scoring of TDP-43 proteinopathy from specific brain regions in 27 academic research centers: associations with Alzheimer's disease and cerebrovascular disease pathologies.
Acta neuropathologica communications
Katsumata Y,Fardo DW,Kukull WA,Nelson PT
Wed Dec 19 00:00:00 EST 2018
Dichotomous scoring of TDP-43 proteinopathy from specific brain regions in 27 academic research centers: associations with Alzheimer's disease and cerebrovascular disease pathologies.
Acta neuropathologica communications
Katsumata Y,Fardo DW,Kukull WA,Nelson PT
Wed Dec 19 00:00:00 EST 2018
Identification of TCERG1 as a new genetic modulator of TDP-43 production in Drosophila.
Acta neuropathologica communications
Pons M,Prieto S,Miguel L,Frebourg T,Campion D,Suñé C,Lecourtois M
Wed Dec 12 00:00:00 EST 2018
Enteroviral Infection Leads to Transactive Response DNA-Binding Protein 43 Pathology in Vivo.
The American journal of pathology
Xue YC,Ruller CM,Fung G,Mohamud Y,Deng H,Liu H,Zhang J,Feuer R,Luo H
Sat Dec 01 00:00:00 EST 2018
TDP-43 pathology in multiple system atrophy: colocalization of TDP-43 and α-synuclein in glial cytoplasmic inclusions.
Neuropathology and applied neurobiology
Koga S,Lin WL,Walton RL,Ross OA,Dickson DW
Sat Dec 01 00:00:00 EST 2018
Liquid Nuclear Condensates Mechanically Sense and Restructure the Genome.
Cell
Shin Y,Chang YC,Lee DSW,Berry J,Sanders DW,Ronceray P,Wingreen NS,Haataja M,Brangwynne CP
Thu Nov 29 00:00:00 EST 2018
mTh1 driven expression of hTDP-43 results in typical ALS/FTLD neuropathological symptoms.
PloS one
Scherz B,Rabl R,Flunkert S,Rohler S,Neddens J,Taub N,Temmel M,Panzenboeck U,Niederkofler V,Zimmermann R,Hutter-Paier B
Mon Nov 26 00:00:00 EST 2018
mTh1 driven expression of hTDP-43 results in typical ALS/FTLD neuropathological symptoms.
PloS one
Scherz B,Rabl R,Flunkert S,Rohler S,Neddens J,Taub N,Temmel M,Panzenboeck U,Niederkofler V,Zimmermann R,Hutter-Paier B
Mon Nov 26 00:00:00 EST 2018
Angiopoietin-1 and ανβ3 integrin peptide promote the therapeutic effects of L-serine in an amyotrophic lateral sclerosis/Parkinsonism dementia complex model.
Aging
Cai HY,Tian KW,Zhang YY,Jiang H,Han S
Sun Nov 25 00:00:00 EST 2018
Age-related deregulation of TDP-43 after stroke enhances NF-κB-mediated inflammation and neuronal damage.
Journal of neuroinflammation
Thammisetty SS,Pedragosa J,Weng YC,Calon F,Planas A,Kriz J
Fri Nov 09 00:00:00 EST 2018
Age-related deregulation of TDP-43 after stroke enhances NF-κB-mediated inflammation and neuronal damage.
Journal of neuroinflammation
Thammisetty SS,Pedragosa J,Weng YC,Calon F,Planas A,Kriz J
Fri Nov 09 00:00:00 EST 2018
Mutant UBQLN2P497H in motor neurons leads to ALS-like phenotypes and defective autophagy in rats.
Acta neuropathologica communications
Chen T,Huang B,Shi X,Gao L,Huang C
Thu Nov 08 00:00:00 EST 2018
Muscle-dominant wild-type TDP-43 expression induces myopathological changes featuring tubular aggregates and TDP-43-positive inclusions.
Experimental neurology
Tawara N,Yamashita S,Kawakami K,Kurashige T,Zhang Z,Tasaki M,Yamamoto Y,Nishikami T,Doki T,Zhang X,Matsuo Y,Kimura E,Tawara A,Maeda Y,Hauschka SD,Maruyama H,Ando Y
Thu Nov 01 00:00:00 EDT 2018
Muscle-dominant wild-type TDP-43 expression induces myopathological changes featuring tubular aggregates and TDP-43-positive inclusions.
Experimental neurology
Tawara N,Yamashita S,Kawakami K,Kurashige T,Zhang Z,Tasaki M,Yamamoto Y,Nishikami T,Doki T,Zhang X,Matsuo Y,Kimura E,Tawara A,Maeda Y,Hauschka SD,Maruyama H,Ando Y
Thu Nov 01 00:00:00 EDT 2018
TDP-43 and RNA form amyloid-like myo-granules in regenerating muscle.
Nature
Vogler TO,Wheeler JR,Nguyen ED,Hughes MP,Britson KA,Lester E,Rao B,Betta ND,Whitney ON,Ewachiw TE,Gomes E,Shorter J,Lloyd TE,Eisenberg DS,Taylor JP,Johnson AM,Olwin BB,Parker R
Thu Nov 01 00:00:00 EDT 2018
TDP-43 and RNA form amyloid-like myo-granules in regenerating muscle.
Nature
Vogler TO,Wheeler JR,Nguyen ED,Hughes MP,Britson KA,Lester E,Rao B,Betta ND,Whitney ON,Ewachiw TE,Gomes E,Shorter J,Lloyd TE,Eisenberg DS,Taylor JP,Johnson AM,Olwin BB,Parker R
Thu Nov 01 00:00:00 EDT 2018
Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly.
Proceedings of the National Academy of Sciences of the United States of America
Sharkey LM,Safren N,Pithadia AS,Gerson JE,Dulchavsky M,Fischer S,Patel R,Lantis G,Ashraf N,Kim JH,Meliki A,Minakawa EN,Barmada SJ,Ivanova MI,Paulson HL
Tue Oct 30 00:00:00 EDT 2018
Aberrant activation of non-coding RNA targets of transcriptional elongation complexes contributes to TDP-43 toxicity.
Nature communications
Chung CY,Berson A,Kennerdell JR,Sartoris A,Unger T,Porta S,Kim HJ,Smith ER,Shilatifard A,Van Deerlin V,Lee VM,Chen-Plotkin A,Bonini NM
Tue Oct 23 00:00:00 EDT 2018
Progranulin reduces insoluble TDP-43 levels, slows down axonal degeneration and prolongs survival in mutant TDP-43 mice.
Molecular neurodegeneration
Beel S,Herdewyn S,Fazal R,De Decker M,Moisse M,Robberecht W,Van Den Bosch L,Van Damme P
Tue Oct 16 00:00:00 EDT 2018
Epigenetic Changes Associated with the Expression of Amyotrophic Lateral Sclerosis (ALS) Causing Genes.
Neuroscience
Masala A,Sanna S,Esposito S,Rassu M,Galioto M,Zinellu A,Carru C,Carrì MT,Iaccarino C,Crosio C
Mon Oct 15 00:00:00 EDT 2018
Epigenetic Changes Associated with the Expression of Amyotrophic Lateral Sclerosis (ALS) Causing Genes.
Neuroscience
Masala A,Sanna S,Esposito S,Rassu M,Galioto M,Zinellu A,Carru C,Carrì MT,Iaccarino C,Crosio C
Mon Oct 15 00:00:00 EDT 2018
Identification and characterization of ubiquitinylation sites in TAR DNA-binding protein of 43 kDa (TDP-43).
The Journal of biological chemistry
Hans F,Eckert M,von Zweydorf F,Gloeckner CJ,Kahle PJ
Fri Oct 12 00:00:00 EDT 2018
Identification and characterization of ubiquitinylation sites in TAR DNA-binding protein of 43 kDa (TDP-43).
The Journal of biological chemistry
Hans F,Eckert M,von Zweydorf F,Gloeckner CJ,Kahle PJ
Fri Oct 12 00:00:00 EDT 2018
Distinct multilevel misregulations of Parkin and PINK1 revealed in cell and animal models of TDP-43 proteinopathy.
Cell death & disease
Sun X,Duan Y,Qin C,Li JC,Duan G,Deng X,Ni J,Cao X,Xiang K,Tian K,Chen CH,Li A,Fang Y
Thu Sep 20 00:00:00 EDT 2018
FTLD/ALS-linked TDP-43 mutations do not alter TDP-43's ability to self-regulate its expression in Drosophila.
Brain research
Miguel L,Avequin T,Pons M,Frebourg T,Campion D,Lecourtois M
Sat Sep 15 00:00:00 EDT 2018
Staufen1 links RNA stress granules and autophagy in a model of neurodegeneration.
Nature communications
Paul S,Dansithong W,Figueroa KP,Scoles DR,Pulst SM
Fri Sep 07 00:00:00 EDT 2018
Poly(ADP-Ribose) Prevents Pathological Phase Separation of TDP-43 by Promoting Liquid Demixing and Stress Granule Localization.
Molecular cell
McGurk L,Gomes E,Guo L,Mojsilovic-Petrovic J,Tran V,Kalb RG,Shorter J,Bonini NM
Thu Sep 06 00:00:00 EDT 2018
Poly(ADP-Ribose) Prevents Pathological Phase Separation of TDP-43 by Promoting Liquid Demixing and Stress Granule Localization.
Molecular cell
McGurk L,Gomes E,Guo L,Mojsilovic-Petrovic J,Tran V,Kalb RG,Shorter J,Bonini NM
Thu Sep 06 00:00:00 EDT 2018
TBK1 Suppresses RIPK1-Driven Apoptosis and Inflammation during Development and in Aging.
Cell
Xu D,Jin T,Zhu H,Chen H,Ofengeim D,Zou C,Mifflin L,Pan L,Amin P,Li W,Shan B,Naito MG,Meng H,Li Y,Pan H,Aron L,Adiconis X,Levin JZ,Yankner BA,Yuan J
Thu Sep 06 00:00:00 EDT 2018
Spreading of Tau Pathology in Sporadic Alzheimer's Disease Along Cortico-cortical Top-Down Connections.
Cerebral cortex (New York, N.Y. : 1991)
Braak H,Del Tredici K
Sat Sep 01 00:00:00 EDT 2018
Transactive Response DNA-Binding Protein 43 Abnormalities after Traumatic Brain Injury.
Journal of neurotrauma
Tan XL,Sun M,Brady RD,Liu S,Llanos R,Cheung S,Wright DK,Casillas-Espinosa PM,Sashindranath M,O'Brien TJ,McDonald SJ,Turner BJ,Shultz SR
Fri Aug 10 00:00:00 EDT 2018
Disruption by SaCas9 Endonuclease of HERV-Kenv, a Retroviral Gene with Oncogenic and Neuropathogenic Potential, Inhibits Molecules Involved in Cancer and Amyotrophic Lateral Sclerosis.
Viruses
Ibba G,Piu C,Uleri E,Serra C,Dolei A
Tue Aug 07 00:00:00 EDT 2018
Primary fibroblasts derived from sporadic amyotrophic lateral sclerosis patients do not show ALS cytological lesions.
Amyotrophic lateral sclerosis & frontotemporal degeneration
Codron P,Cassereau J,Vourc'h P,Veyrat-Durebex C,Blasco H,Kane S,Procaccio V,Letournel F,Verny C,Lenears G,Reynier P,Chevrollier A
Wed Aug 01 00:00:00 EDT 2018
Potentiating Hsp104 activity via phosphomimetic mutations in the middle domain.
FEMS yeast research
Tariq A,Lin J,Noll MM,Torrente MP,Mack KL,Murillo OH,Jackrel ME,Shorter J
Wed Aug 01 00:00:00 EDT 2018
Potentiating Hsp104 activity via phosphomimetic mutations in the middle domain.
FEMS yeast research
Tariq A,Lin J,Noll MM,Torrente MP,Mack KL,Murillo OH,Jackrel ME,Shorter J
Wed Aug 01 00:00:00 EDT 2018
Analysis of endocannabinoid receptors and enzymes in the post-mortem motor cortex and spinal cord of amyotrophic lateral sclerosis patients.
Amyotrophic lateral sclerosis & frontotemporal degeneration
Espejo-Porras F,Fernández-Ruiz J,de Lago E
Wed Aug 01 00:00:00 EDT 2018
Unexpected similarities between C9ORF72 and sporadic forms of ALS/FTD suggest a common disease mechanism.
eLife
Conlon EG,Fagegaltier D,Agius P,Davis-Porada J,Gregory J,Hubbard I,Kang K,Kim D,Phatnani H,Shneider NA,Manley JL
Fri Jul 13 00:00:00 EDT 2018
The age of onset and evolution of Braak tangle stage and Thal amyloid pathology of Alzheimer's disease in individuals with Down syndrome.
Acta neuropathologica communications
Davidson YS,Robinson A,Prasher VP,Mann DMA
Wed Jul 04 00:00:00 EDT 2018
Motor neuron differentiation of iPSCs obtained from peripheral blood of a mutant TARDBP ALS patient.
Stem cell research
Bossolasco P,Sassone F,Gumina V,Peverelli S,Garzo M,Silani V
Sun Jul 01 00:00:00 EDT 2018
Neuron-Specific HuR-Deficient Mice Spontaneously Develop Motor Neuron Disease.
Journal of immunology (Baltimore, Md. : 1950)
Sun K,Li X,Chen X,Bai Y,Zhou G,Kokiko-Cochran ON,Lamb B,Hamilton TA,Lin CY,Lee YS,Herjan T
Sun Jul 01 00:00:00 EDT 2018
Accumulation of poly(A) RNA in nuclear granules enriched in Sam68 in motor neurons from the SMNΔ7 mouse model of SMA.
Scientific reports
Narcís JO,Tapia O,Tarabal O,Piedrafita L,Calderó J,Berciano MT,Lafarga M
Mon Jun 25 00:00:00 EDT 2018
DCTN1 F52L mutation case of Perry syndrome with progressive supranuclear palsy-like tauopathy.
Parkinsonism & related disorders
Honda H,Sasagasako N,Shen C,Shijo M,Hamasaki H,Suzuki SO,Tsuboi Y,Fujii N,Iwaki T
Fri Jun 01 00:00:00 EDT 2018
Frontotemporal dementia with trans-activation response DNA-binding protein 43 presenting with catatonic syndrome.
Neuropathology : official journal of the Japanese Society of Neuropathology
Watanabe R,Kawakami I,Onaya M,Higashi S,Arai N,Akiyama H,Hasegawa M,Arai T
Fri Jun 01 00:00:00 EDT 2018
Protective paraspeckle hyper-assembly downstream of TDP-43 loss of function in amyotrophic lateral sclerosis.
Molecular neurodegeneration
Shelkovnikova TA,Kukharsky MS,An H,Dimasi P,Alexeeva S,Shabir O,Heath PR,Buchman VL
Fri Jun 01 00:00:00 EDT 2018
Mice with endogenous TDP-43 mutations exhibit gain of splicing function and characteristics of amyotrophic lateral sclerosis.
The EMBO journal
Fratta P,Sivakumar P,Humphrey J,Lo K,Ricketts T,Oliveira H,Brito-Armas JM,Kalmar B,Ule A,Yu Y,Birsa N,Bodo C,Collins T,Conicella AE,Mejia Maza A,Marrero-Gagliardi A,Stewart M,Mianne J,Corrochano S,Emmett W,Codner G,Groves M,Fukumura R,Gondo Y,Lythgoe M,Pauws E,Peskett E,Stanier P,Teboul L,Hallegger M,Calvo A,Chiò A,Isaacs AM,Fawzi NL,Wang E,Housman DE,Baralle F,Greensmith L,Buratti E,Plagnol V,Fisher EM,Acevedo-Arozena A
Fri Jun 01 00:00:00 EDT 2018
Mice with endogenous TDP-43 mutations exhibit gain of splicing function and characteristics of amyotrophic lateral sclerosis.
The EMBO journal
Fratta P,Sivakumar P,Humphrey J,Lo K,Ricketts T,Oliveira H,Brito-Armas JM,Kalmar B,Ule A,Yu Y,Birsa N,Bodo C,Collins T,Conicella AE,Mejia Maza A,Marrero-Gagliardi A,Stewart M,Mianne J,Corrochano S,Emmett W,Codner G,Groves M,Fukumura R,Gondo Y,Lythgoe M,Pauws E,Peskett E,Stanier P,Teboul L,Hallegger M,Calvo A,Chiò A,Isaacs AM,Fawzi NL,Wang E,Housman DE,Baralle F,Greensmith L,Buratti E,Plagnol V,Fisher EM,Acevedo-Arozena A
Fri Jun 01 00:00:00 EDT 2018
Diabetes is Not Associated with Alzheimer's Disease Neuropathology.
Journal of Alzheimer's disease : JAD
Dos Santos Matioli MNP,Suemoto CK,Rodriguez RD,Farias DS,da Silva MM,Leite REP,Ferretti-Rebustini REL,Farfel JM,Pasqualucci CA,Jacob Filho W,Arvanitakis Z,Naslavsky MS,Zatz M,Grinberg LT,Nitrini R
Wed May 30 00:00:00 EDT 2018
TDP-43 regulation of stress granule dynamics in neurodegenerative disease-relevant cell types.
Scientific reports
Khalfallah Y,Kuta R,Grasmuck C,Prat A,Durham HD,Vande Velde C
Tue May 15 00:00:00 EDT 2018
TDP-43 regulation of stress granule dynamics in neurodegenerative disease-relevant cell types.
Scientific reports
Khalfallah Y,Kuta R,Grasmuck C,Prat A,Durham HD,Vande Velde C
Tue May 15 00:00:00 EDT 2018
Active nuclear import and passive nuclear export are the primary determinants of TDP-43 localization.
Scientific reports
Pinarbasi ES,Cağatay T,Fung HYJ,Li YC,Chook YM,Thomas PJ
Fri May 04 00:00:00 EDT 2018
TDP-43 regulates the alternative splicing of hnRNP A1 to yield an aggregation-prone variant in amyotrophic lateral sclerosis.
Brain : a journal of neurology
Deshaies JE,Shkreta L,Moszczynski AJ,Sidibé H,Semmler S,Fouillen A,Bennett ER,Bekenstein U,Destroismaisons L,Toutant J,Delmotte Q,Volkening K,Stabile S,Aulas A,Khalfallah Y,Soreq H,Nanci A,Strong MJ,Chabot B,Vande Velde C
Tue May 01 00:00:00 EDT 2018
Essential role of hippocampal noradrenaline in the regulation of spatial working memory and TDP-43 tissue pathology.
The Journal of comparative neurology
Pintus R,Riggi M,Cannarozzo C,Valeri A,de Leo G,Romano M,Gulino R,Leanza G
Tue May 01 00:00:00 EDT 2018
Neurodegenerative Disease Proteinopathies Are Connected to Distinct Histone Post-translational Modification Landscapes.
ACS chemical neuroscience
Chen K,Bennett SA,Rana N,Yousuf H,Said M,Taaseen S,Mendo N,Meltser SM,Torrente MP
Wed Apr 18 00:00:00 EDT 2018
Phosphorylated TDP-43 (pTDP-43) aggregates in the axial skeletal muscle of patients with sporadic and familial amyotrophic lateral sclerosis.
Acta neuropathologica communications
Cykowski MD,Powell SZ,Appel JW,Arumanayagam AS,Rivera AL,Appel SH
Fri Apr 13 00:00:00 EDT 2018
Microglial and Neuronal TDP-43 Pathology in Anti-IgLON5-Related Tauopathy.
Journal of Alzheimer's disease : JAD
Cagnin A,Mariotto S,Fiorini M,Gaule M,Bonetto N,Tagliapietra M,Buratti E,Zanusso G,Ferrari S,Monaco S
Fri Apr 06 00:00:00 EDT 2018
Microglial and Neuronal TDP-43 Pathology in Anti-IgLON5-Related Tauopathy.
Journal of Alzheimer's disease : JAD
Cagnin A,Mariotto S,Fiorini M,Gaule M,Bonetto N,Tagliapietra M,Buratti E,Zanusso G,Ferrari S,Monaco S
Fri Apr 06 00:00:00 EDT 2018
RNA and Protein Interactors with TDP-43 in Human Spinal-Cord Lysates in Amyotrophic Lateral Sclerosis.
Journal of proteome research
Volkening K,Keller BA,Leystra-Lantz C,Strong MJ
Fri Apr 06 00:00:00 EDT 2018
Lymphoblastoid cell lines as a model to understand amyotrophic lateral sclerosis disease mechanisms.
Disease models & mechanisms
Pansarasa O,Bordoni M,Drufuca L,Diamanti L,Sproviero D,Trotti R,Bernuzzi S,La Salvia S,Gagliardi S,Ceroni M,Cereda C
Mon Mar 26 00:00:00 EDT 2018
Lymphoblastoid cell lines as a model to understand amyotrophic lateral sclerosis disease mechanisms.
Disease models & mechanisms
Pansarasa O,Bordoni M,Drufuca L,Diamanti L,Sproviero D,Trotti R,Bernuzzi S,La Salvia S,Gagliardi S,Ceroni M,Cereda C
Mon Mar 26 00:00:00 EDT 2018
TDP43 nuclear export and neurodegeneration in models of amyotrophic lateral sclerosis and frontotemporal dementia.
Scientific reports
Archbold HC,Jackson KL,Arora A,Weskamp K,Tank EM,Li X,Miguez R,Dayton RD,Tamir S,Klein RL,Barmada SJ
Thu Mar 15 00:00:00 EDT 2018
TDP43 nuclear export and neurodegeneration in models of amyotrophic lateral sclerosis and frontotemporal dementia.
Scientific reports
Archbold HC,Jackson KL,Arora A,Weskamp K,Tank EM,Li X,Miguez R,Dayton RD,Tamir S,Klein RL,Barmada SJ
Thu Mar 15 00:00:00 EDT 2018
Zc3h13 Regulates Nuclear RNA m6A Methylation and Mouse Embryonic Stem Cell Self-Renewal.
Molecular cell
Wen J,Lv R,Ma H,Shen H,He C,Wang J,Jiao F,Liu H,Yang P,Tan L,Lan F,Shi YG,He C,Shi Y,Diao J
Thu Mar 15 00:00:00 EDT 2018
Overlapping Role of SCYL1 and SCYL3 in Maintaining Motor Neuron Viability.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Kuliyev E,Gingras S,Guy CS,Howell S,Vogel P,Pelletier S
Wed Mar 07 00:00:00 EST 2018
A single N-terminal phosphomimic disrupts TDP-43 polymerization, phase separation, and RNA splicing.
The EMBO journal
Wang A,Conicella AE,Schmidt HB,Martin EW,Rhoads SN,Reeb AN,Nourse A,Ramirez Montero D,Ryan VH,Rohatgi R,Shewmaker F,Naik MT,Mittag T,Ayala YM,Fawzi NL
Thu Mar 01 00:00:00 EST 2018
Tau Rather than TDP-43 Proteins are Potential Cerebrospinal Fluid Biomarkers for Frontotemporal Lobar Degeneration Subtypes: A Pilot Study.
Journal of Alzheimer's disease : JAD
Kuiperij HB,Versleijen AA,Beenes M,Verwey NA,Benussi L,Paterlini A,Binetti G,Teunissen CE,Raaphorst J,Schelhaas HJ,Küsters B,Pijnenburg YA,Ghidoni R,Verbeek MM
Thu Feb 22 00:00:00 EST 2018
Behavioral defects in a DCTN1G71A transgenic mouse model of Perry syndrome.
Neuroscience letters
Mishima T,Deshimaru M,Watanabe T,Kubota K,Kinoshita-Kawada M,Yuasa-Kawada J,Takasaki K,Uehara Y,Jinno S,Iwasaki K,Tsuboi Y
Wed Feb 14 00:00:00 EST 2018
Cerebral ischemia induces the aggregation of proteins linked to neurodegenerative diseases.
Scientific reports
Kahl A,Blanco I,Jackman K,Baskar J,Milaganur Mohan H,Rodney-Sandy R,Zhang S,Iadecola C,Hochrainer K
Fri Feb 09 00:00:00 EST 2018
Citrullination of RGG Motifs in FET Proteins by PAD4 Regulates Protein Aggregation and ALS Susceptibility.
Cell reports
Tanikawa C,Ueda K,Suzuki A,Iida A,Nakamura R,Atsuta N,Tohnai G,Sobue G,Saichi N,Momozawa Y,Kamatani Y,Kubo M,Yamamoto K,Nakamura Y,Matsuda K
Tue Feb 06 00:00:00 EST 2018
Mutation in the RRM2 domain of TDP-43 in Amyotrophic Lateral Sclerosis with rapid progression associated with ubiquitin positive aggregates in cultured motor neurons.
Amyotrophic lateral sclerosis & frontotemporal degeneration
Maurel C,Madji-Hounoum B,Thepault RA,Marouillat S,Brulard C,Danel-Brunaud V,Camdessanche JP,Blasco H,Corcia P,Andres CR,Vourc'h P
Thu Feb 01 00:00:00 EST 2018
Mutation in the RRM2 domain of TDP-43 in Amyotrophic Lateral Sclerosis with rapid progression associated with ubiquitin positive aggregates in cultured motor neurons.
Amyotrophic lateral sclerosis & frontotemporal degeneration
Maurel C,Madji-Hounoum B,Thepault RA,Marouillat S,Brulard C,Danel-Brunaud V,Camdessanche JP,Blasco H,Corcia P,Andres CR,Vourc'h P
Thu Feb 01 00:00:00 EST 2018
Protein Phosphatase 1 dephosphorylates TDP-43 and suppresses its function in tau exon 10 inclusion.
FEBS letters
Gu J,Wang W,Miao S,Chen F,Wu F,Hu W,Iqbal K,Gong CX,Liu F
Thu Feb 01 00:00:00 EST 2018
Multiple Proteinopathies in Familial ALS Cases With Optineurin Mutations.
Journal of neuropathology and experimental neurology
Ayaki T,Ito H,Komure O,Kamada M,Nakamura M,Wate R,Kusaka H,Yamaguchi Y,Li F,Kawakami H,Urushitani M,Takahashi R
Thu Feb 01 00:00:00 EST 2018
Angiogenin Prevents Progranulin A9D Mutation-Induced Neuronal-Like Cell Apoptosis Through Cleaving tRNAs into tiRNAs.
Molecular neurobiology
Li S,Chen Y,Sun D,Bai R,Gao X,Yang Y,Sheng J,Xu Z
Thu Feb 01 00:00:00 EST 2018
Mitochondrial abnormalities and disruption of the neuromuscular junction precede the clinical phenotype and motor neuron loss in hFUSWT transgenic mice.
Human molecular genetics
So E,Mitchell JC,Memmi C,Chennell G,Vizcay-Barrena G,Allison L,Shaw CE,Vance C
Thu Feb 01 00:00:00 EST 2018
Characterization of Dopaminergic System in the Striatum of Young Adult Park2-/- Knockout Rats.
Scientific reports
Gemechu JM,Sharma A,Yu D,Xie Y,Merkel OM,Moszczynska A
Wed Jan 24 00:00:00 EST 2018
Characterization of Dopaminergic System in the Striatum of Young Adult Park2-/- Knockout Rats.
Scientific reports
Gemechu JM,Sharma A,Yu D,Xie Y,Merkel OM,Moszczynska A
Wed Jan 24 00:00:00 EST 2018
Progression of tau pathology within cholinergic nucleus basalis neurons in chronic traumatic encephalopathy: A chronic effects of neurotrauma consortium study.
Brain injury
Mufson EJ,Perez SE,Nadeem M,Mahady L,Kanaan NM,Abrahamson EE,Ikonomovic MD,Crawford F,Alvarez V,Stein T,McKee AC
Wed Jan 03 00:00:00 EST 2018
Anterior Cingulate Cortex TDP-43 Pathology in Sporadic Amyotrophic Lateral Sclerosis.
Journal of neuropathology and experimental neurology
Braak H,Del Tredici K
Mon Jan 01 00:00:00 EST 2018
Alzheimer neuropathology without frontotemporal lobar degeneration hallmarks (TAR DNA-binding protein 43 inclusions) in missense progranulin mutation Cys139Arg.
Brain pathology (Zurich, Switzerland)
Redaelli V,Rossi G,Maderna E,Kovacs GG,Piccoli E,Caroppo P,Cacciatore F,Spinello S,Grisoli M,Sozzi G,Salmaggi A,Tagliavini F,Giaccone G
Mon Jan 01 00:00:00 EST 2018
A proteomic network approach across the ALS-FTD disease spectrum resolves clinical phenotypes and genetic vulnerability in human brain.
EMBO molecular medicine
Umoh ME,Dammer EB,Dai J,Duong DM,Lah JJ,Levey AI,Gearing M,Glass JD,Seyfried NT
Mon Jan 01 00:00:00 EST 2018
Repetitive Mild Closed Head Injury Alters Protein Expression and Dendritic Complexity in a Mouse Model.
Journal of neurotrauma
Saykally JN,Ratliff WA,Keeley KL,Pick CG,Mervis RF,Citron BA
Mon Jan 01 00:00:00 EST 2018
TDP-43 proteolysis is associated with astrocyte reactivity after traumatic brain injury in rodents.
Journal of neuroimmunology
Huang CY,Lee YC,Li PC,Liliang PC,Lu K,Wang KW,Chang LC,Shiu LY,Chen MF,Sun YT,Wang HK
Fri Dec 15 00:00:00 EST 2017
Alcohol-abuse drug disulfiram targets cancer via p97 segregase adaptor NPL4.
Nature
Skrott Z,Mistrik M,Andersen KK,Friis S,Majera D,Gursky J,Ozdian T,Bartkova J,Turi Z,Moudry P,Kraus M,Michalova M,Vaclavkova J,Dzubak P,Vrobel I,Pouckova P,Sedlacek J,Miklovicova A,Kutt A,Li J,Mattova J,Driessen C,Dou QP,Olsen J,Hajduch M,Cvek B,Deshaies RJ,Bartek J
Thu Dec 14 00:00:00 EST 2017
Endocytosis regulates TDP-43 toxicity and turnover.
Nature communications
Liu G,Coyne AN,Pei F,Vaughan S,Chaung M,Zarnescu DC,Buchan JR
Tue Dec 12 00:00:00 EST 2017
Endocytosis regulates TDP-43 toxicity and turnover.
Nature communications
Liu G,Coyne AN,Pei F,Vaughan S,Chaung M,Zarnescu DC,Buchan JR
Tue Dec 12 00:00:00 EST 2017
Clinical and neuropathological features of ALS/FTD with TIA1 mutations.
Acta neuropathologica communications
Hirsch-Reinshagen V,Pottier C,Nicholson AM,Baker M,Hsiung GR,Krieger C,Sengdy P,Boylan KB,Dickson DW,Mesulam M,Weintraub S,Bigio E,Zinman L,Keith J,Rogaeva E,Zivkovic SA,Lacomis D,Taylor JP,Rademakers R,Mackenzie IRA
Thu Dec 07 00:00:00 EST 2017
De novo design of RNA-binding proteins with a prion-like domain related to ALS/FTD proteinopathies.
Scientific reports
Mitsuhashi K,Ito D,Mashima K,Oyama M,Takahashi S,Suzuki N
Mon Dec 04 00:00:00 EST 2017
TDP-43 Promotes Neurodegeneration by Impairing Chromatin Remodeling.
Current biology : CB
Berson A,Sartoris A,Nativio R,Van Deerlin V,Toledo JB,Porta S,Liu S,Chung CY,Garcia BA,Lee VM,Trojanowski JQ,Johnson FB,Berger SL,Bonini NM
Mon Dec 04 00:00:00 EST 2017
TDP-43 Promotes Neurodegeneration by Impairing Chromatin Remodeling.
Current biology : CB
Berson A,Sartoris A,Nativio R,Van Deerlin V,Toledo JB,Porta S,Liu S,Chung CY,Garcia BA,Lee VM,Trojanowski JQ,Johnson FB,Berger SL,Bonini NM
Mon Dec 04 00:00:00 EST 2017
Imbalanced expression of polycistronic miRNA in acute myeloid leukemia.
International journal of hematology
Kotaki R,Higuchi H,Ogiya D,Katahira Y,Kurosaki N,Yukihira N,Ogata J,Yamamoto H,Mohamad Alba S,Azhim A,Kitajima T,Inoue S,Morishita K,Ono K,Koyama-Nasu R,Kotani A
Fri Dec 01 00:00:00 EST 2017
An intronic variation in SLC52A1 causes exon skipping and transient riboflavin-responsive multiple acyl-CoA dehydrogenation deficiency.
Molecular genetics and metabolism
Mosegaard S,Bruun GH,Flyvbjerg KF,Bliksrud YT,Gregersen N,Dembic M,Annexstad E,Tangeraas T,Olsen RKJ,Andresen BS
Fri Dec 01 00:00:00 EST 2017
Regional correlations between [11C]PIB PET and post-mortem burden of amyloid-beta pathology in a diverse neuropathological cohort.
NeuroImage. Clinical
Seo SW,Ayakta N,Grinberg LT,Villeneuve S,Lehmann M,Reed B,DeCarli C,Miller BL,Rosen HJ,Boxer AL,O'Neil JP,Jin LW,Seeley WW,Jagust WJ,Rabinovici GD
Fri Nov 17 00:00:00 EST 2017
TDP-43 misexpression causes defects in dendritic growth.
Scientific reports
Herzog JJ,Deshpande M,Shapiro L,Rodal AA,Paradis S
Wed Nov 15 00:00:00 EST 2017
Clusterin protects neurons against intracellular proteotoxicity.
Acta neuropathologica communications
Gregory JM,Whiten DR,Brown RA,Barros TP,Kumita JR,Yerbury JJ,Satapathy S,McDade K,Smith C,Luheshi LM,Dobson CM,Wilson MR
Tue Nov 07 00:00:00 EST 2017
TDP-43 accelerates age-dependent degeneration of interneurons.
Scientific reports
Tsuiji H,Inoue I,Takeuchi M,Furuya A,Yamakage Y,Watanabe S,Koike M,Hattori M,Yamanaka K
Thu Nov 02 00:00:00 EDT 2017
TDP-43 accelerates age-dependent degeneration of interneurons.
Scientific reports
Tsuiji H,Inoue I,Takeuchi M,Furuya A,Yamakage Y,Watanabe S,Koike M,Hattori M,Yamanaka K
Thu Nov 02 00:00:00 EDT 2017
Platelet phosphorylated TDP-43: an exploratory study for a peripheral surrogate biomarker development for Alzheimer's disease.
Future science OA
Wilhite R,Sage JM,Bouzid A,Primavera T,Agbas A
Wed Nov 01 00:00:00 EDT 2017
Dysregulation of chromatin remodelling complexes in amyotrophic lateral sclerosis.
Human molecular genetics
Tibshirani M,Zhao B,Gentil BJ,Minotti S,Marques C,Keith J,Rogaeva E,Zinman L,Rouaux C,Robertson J,Durham HD
Wed Nov 01 00:00:00 EDT 2017
Disulfide cross-linked multimers of TDP-43 and spinal motoneuron loss in a TDP-43A315T ALS/FTD mouse model.
Scientific reports
Bargsted L,Medinas DB,Martínez Traub F,Rozas P,Muñoz N,Nassif M,Jerez C,Catenaccio A,Court FA,Hetz C,Matus S
Fri Oct 27 00:00:00 EDT 2017
Disulfide cross-linked multimers of TDP-43 and spinal motoneuron loss in a TDP-43A315T ALS/FTD mouse model.
Scientific reports
Bargsted L,Medinas DB,Martínez Traub F,Rozas P,Muñoz N,Nassif M,Jerez C,Catenaccio A,Court FA,Hetz C,Matus S
Fri Oct 27 00:00:00 EDT 2017
Identification of functional tetramolecular RNA G-quadruplexes derived from transfer RNAs.
Nature communications
Lyons SM,Gudanis D,Coyne SM,Gdaniec Z,Ivanov P
Tue Oct 24 00:00:00 EDT 2017
Cis P-tau is induced in clinical and preclinical brain injury and contributes to post-injury sequelae.
Nature communications
Albayram O,Kondo A,Mannix R,Smith C,Tsai CY,Li C,Herbert MK,Qiu J,Monuteaux M,Driver J,Yan S,Gormley W,Puccio AM,Okonkwo DO,Lucke-Wold B,Bailes J,Meehan W,Zeidel M,Lu KP,Zhou XZ
Tue Oct 17 00:00:00 EDT 2017
Kinase Inhibitor Screening Identifies Cyclin-Dependent Kinases and Glycogen Synthase Kinase 3 as Potential Modulators of TDP-43 Cytosolic Accumulation during Cell Stress.
PloS one
Moujalled D,James JL,Parker SJ,Lidgerwood GE,Duncan C,Meyerowitz J,Nonaka T,Hasegawa M,Kanninen KM,Grubman A,Liddell JR,Crouch PJ,White AR
Mon Oct 16 00:00:00 EDT 2017
Nuclear poly(A) binding protein 1 (PABPN1) and Matrin3 interact in muscle cells and regulate RNA processing.
Nucleic acids research
Banerjee A,Vest KE,Pavlath GK,Corbett AH
Fri Oct 13 00:00:00 EDT 2017
Novel miR-b2122 regulates several ALS-related RNA-binding proteins.
Molecular brain
Hawley ZCE,Campos-Melo D,Strong MJ
Mon Oct 02 00:00:00 EDT 2017
The Effect of Different Types of Nanoparticles on FUS and TDP-43 Solubility and Subcellular Localization.
Neurotoxicity research
Lojk J,Prpar Mihevc S,Bregar VB,Pavlin M,Rogelj B
Sun Oct 01 00:00:00 EDT 2017
The Effect of Different Types of Nanoparticles on FUS and TDP-43 Solubility and Subcellular Localization.
Neurotoxicity research
Lojk J,Prpar Mihevc S,Bregar VB,Pavlin M,Rogelj B
Sun Oct 01 00:00:00 EDT 2017
Truncation of the TAR DNA-binding protein 43 is not a prerequisite for cytoplasmic relocalization, and is suppressed by caspase inhibition and by introduction of the A90V sequence variant.
PloS one
Wobst HJ,Delsing L,Brandon NJ,Moss SJ
Wed Sep 06 00:00:00 EDT 2017
Truncation of the TAR DNA-binding protein 43 is not a prerequisite for cytoplasmic relocalization, and is suppressed by caspase inhibition and by introduction of the A90V sequence variant.
PloS one
Wobst HJ,Delsing L,Brandon NJ,Moss SJ
Wed Sep 06 00:00:00 EDT 2017
Calpain-Dependent Degradation of Nucleoporins Contributes to Motor Neuron Death in a Mouse Model of Chronic Excitotoxicity.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Sugiyama K,Aida T,Nomura M,Takayanagi R,Zeilhofer HU,Tanaka K
Wed Sep 06 00:00:00 EDT 2017
Calpain-Dependent Degradation of Nucleoporins Contributes to Motor Neuron Death in a Mouse Model of Chronic Excitotoxicity.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Sugiyama K,Aida T,Nomura M,Takayanagi R,Zeilhofer HU,Tanaka K
Wed Sep 06 00:00:00 EDT 2017
Splicing factors act as genetic modulators of TDP-43 production in a new autoregulatory TDP-43 Drosophila model.
Human molecular genetics
Pons M,Miguel L,Miel C,Avequin T,Juge F,Frebourg T,Campion D,Lecourtois M
Fri Sep 01 00:00:00 EDT 2017
Traumatic Brain Injury Results in Cellular, Structural and Functional Changes Resembling Motor Neuron Disease.
Cerebral cortex (New York, N.Y. : 1991)
Wright DK,Liu S,van der Poel C,McDonald SJ,Brady RD,Taylor L,Yang L,Gardner AJ,Ordidge R,O'Brien TJ,Johnston LA,Shultz SR
Fri Sep 01 00:00:00 EDT 2017
Amygdala TDP-43 Pathology in Frontotemporal Lobar Degeneration and Motor Neuron Disease.
Journal of neuropathology and experimental neurology
Takeda T,Seilhean D,Le Ber I,Millecamps S,Sazdovitch V,Kitagawa K,Uchihara T,Duyckaerts C
Fri Sep 01 00:00:00 EDT 2017
Pathologic Involvement of Glutamatergic Striatal Inputs From the Cortices in TAR DNA-Binding Protein 43 kDa-Related Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis.
Journal of neuropathology and experimental neurology
Riku Y,Watanabe H,Yoshida M,Mimuro M,Iwasaki Y,Masuda M,Ishigaki S,Katsuno M,Sobue G
Fri Sep 01 00:00:00 EDT 2017
Neurotrophic effects of progranulin in vivo in reversing motor neuron defects caused by over or under expression of TDP-43 or FUS.
PloS one
Chitramuthu BP,Kay DG,Bateman A,Bennett HP
Mon Aug 28 00:00:00 EDT 2017
TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics.
Neuron
Mackenzie IR,Nicholson AM,Sarkar M,Messing J,Purice MD,Pottier C,Annu K,Baker M,Perkerson RB,Kurti A,Matchett BJ,Mittag T,Temirov J,Hsiung GR,Krieger C,Murray ME,Kato M,Fryer JD,Petrucelli L,Zinman L,Weintraub S,Mesulam M,Keith J,Zivkovic SA,Hirsch-Reinshagen V,Roos RP,Züchner S,Graff-Radford NR,Petersen RC,Caselli RJ,Wszolek ZK,Finger E,Lippa C,Lacomis D,Stewart H,Dickson DW,Kim HJ,Rogaeva E,Bigio E,Boylan KB,Taylor JP,Rademakers R
Wed Aug 16 00:00:00 EDT 2017
TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics.
Neuron
Mackenzie IR,Nicholson AM,Sarkar M,Messing J,Purice MD,Pottier C,Annu K,Baker M,Perkerson RB,Kurti A,Matchett BJ,Mittag T,Temirov J,Hsiung GR,Krieger C,Murray ME,Kato M,Fryer JD,Petrucelli L,Zinman L,Weintraub S,Mesulam M,Keith J,Zivkovic SA,Hirsch-Reinshagen V,Roos RP,Züchner S,Graff-Radford NR,Petersen RC,Caselli RJ,Wszolek ZK,Finger E,Lippa C,Lacomis D,Stewart H,Dickson DW,Kim HJ,Rogaeva E,Bigio E,Boylan KB,Taylor JP,Rademakers R
Wed Aug 16 00:00:00 EDT 2017
GPNMB ameliorates mutant TDP-43-induced motor neuron cell death.
Journal of neuroscience research
Nagahara Y,Shimazawa M,Ohuchi K,Ito J,Takahashi H,Tsuruma K,Kakita A,Hara H
Tue Aug 01 00:00:00 EDT 2017
Modelling FUS Mislocalisation in an In Vitro Model of Innervated Human Muscle.
Journal of molecular neuroscience : MN
Prpar Mihevc S,Pavlin M,Darovic S,Živin M,Podbregar M,Rogelj B,Mars T
Tue Aug 01 00:00:00 EDT 2017
Perry Syndrome: A Distinctive Type of TDP-43 Proteinopathy.
Journal of neuropathology and experimental neurology
Mishima T,Koga S,Lin WL,Kasanuki K,Castanedes-Casey M,Wszolek ZK,Oh SJ,Tsuboi Y,Dickson DW
Tue Aug 01 00:00:00 EDT 2017
Anti-AMPA GluA3 antibodies in Frontotemporal dementia: a new molecular target.
Scientific reports
Borroni B,Stanic J,Verpelli C,Mellone M,Bonomi E,Alberici A,Bernasconi P,Culotta L,Zianni E,Archetti S,Manes M,Gazzina S,Ghidoni R,Benussi L,Stuani C,Di Luca M,Sala C,Buratti E,Padovani A,Gardoni F
Thu Jul 27 00:00:00 EDT 2017
Major hnRNP proteins act as general TDP-43 functional modifiers both in Drosophila and human neuronal cells.
Nucleic acids research
Appocher C,Mohagheghi F,Cappelli S,Stuani C,Romano M,Feiguin F,Buratti E
Thu Jul 27 00:00:00 EDT 2017
Major hnRNP proteins act as general TDP-43 functional modifiers both in Drosophila and human neuronal cells.
Nucleic acids research
Appocher C,Mohagheghi F,Cappelli S,Stuani C,Romano M,Feiguin F,Buratti E
Thu Jul 27 00:00:00 EDT 2017
TDP-43 Depletion in Microglia Promotes Amyloid Clearance but Also Induces Synapse Loss.
Neuron
Paolicelli RC,Jawaid A,Henstridge CM,Valeri A,Merlini M,Robinson JL,Lee EB,Rose J,Appel S,Lee VM,Trojanowski JQ,Spires-Jones T,Schulz PE,Rajendran L
Wed Jul 19 00:00:00 EDT 2017
TDP-43 Depletion in Microglia Promotes Amyloid Clearance but Also Induces Synapse Loss.
Neuron
Paolicelli RC,Jawaid A,Henstridge CM,Valeri A,Merlini M,Robinson JL,Lee EB,Rose J,Appel S,Lee VM,Trojanowski JQ,Spires-Jones T,Schulz PE,Rajendran L
Wed Jul 19 00:00:00 EDT 2017
Acetylation-induced TDP-43 pathology is suppressed by an HSF1-dependent chaperone program.
Nature communications
Wang P,Wander CM,Yuan CX,Bereman MS,Cohen TJ
Wed Jul 19 00:00:00 EDT 2017
Acetylation-induced TDP-43 pathology is suppressed by an HSF1-dependent chaperone program.
Nature communications
Wang P,Wander CM,Yuan CX,Bereman MS,Cohen TJ
Wed Jul 19 00:00:00 EDT 2017
Point mutations in the N-terminal domain of transactive response DNA-binding protein 43 kDa (TDP-43) compromise its stability, dimerization, and functions.
The Journal of biological chemistry
Mompeán M,Romano V,Pantoja-Uceda D,Stuani C,Baralle FE,Buratti E,Laurents DV
Fri Jul 14 00:00:00 EDT 2017
Point mutations in the N-terminal domain of transactive response DNA-binding protein 43 kDa (TDP-43) compromise its stability, dimerization, and functions.
The Journal of biological chemistry
Mompeán M,Romano V,Pantoja-Uceda D,Stuani C,Baralle FE,Buratti E,Laurents DV
Fri Jul 14 00:00:00 EDT 2017
Misfolded SOD1 is not a primary component of sporadic ALS.
Acta neuropathologica
Da Cruz S,Bui A,Saberi S,Lee SK,Stauffer J,McAlonis-Downes M,Schulte D,Pizzo DP,Parone PA,Cleveland DW,Ravits J
Sat Jul 01 00:00:00 EDT 2017
Dcp1a and GW182 Induce Distinct Cellular Aggregates and Have Different Effects on microRNA Pathway.
DNA and cell biology
Wang X,Chang L,Wang H,Su A,Wu Z
Sat Jul 01 00:00:00 EDT 2017
Expansion of the classification of FTLD-TDP: distinct pathology associated with rapidly progressive frontotemporal degeneration.
Acta neuropathologica
Lee EB,Porta S,Michael Baer G,Xu Y,Suh E,Kwong LK,Elman L,Grossman M,Lee VM,Irwin DJ,Van Deerlin VM,Trojanowski JQ
Sat Jul 01 00:00:00 EDT 2017
TDP-43 expression influences amyloidβ plaque deposition and tau aggregation.
Neurobiology of disease
Davis SA,Gan KA,Dowell JA,Cairns NJ,Gitcho MA
Sat Jul 01 00:00:00 EDT 2017
TDP-43 expression influences amyloidβ plaque deposition and tau aggregation.
Neurobiology of disease
Davis SA,Gan KA,Dowell JA,Cairns NJ,Gitcho MA
Sat Jul 01 00:00:00 EDT 2017
Heterogeneous ribonuclear protein E2 (hnRNP E2) is associated with TDP-43-immunoreactive neurites in Semantic Dementia but not with other TDP-43 pathological subtypes of Frontotemporal Lobar Degeneration.
Acta neuropathologica communications
Davidson YS,Robinson AC,Flood L,Rollinson S,Benson BC,Asi YT,Richardson A,Jones M,Snowden JS,Pickering-Brown S,Lashley T,Mann DMA
Fri Jun 30 00:00:00 EDT 2017
Functional and dynamic polymerization of the ALS-linked protein TDP-43 antagonizes its pathologic aggregation.
Nature communications
Afroz T,Hock EM,Ernst P,Foglieni C,Jambeau M,Gilhespy LAB,Laferriere F,Maniecka Z,Plückthun A,Mittl P,Paganetti P,Allain FHT,Polymenidou M
Thu Jun 29 00:00:00 EDT 2017
Enhancing Mitofusin/Marf ameliorates neuromuscular dysfunction in Drosophila models of TDP-43 proteinopathies.
Neurobiology of aging
Khalil B,Cabirol-Pol MJ,Miguel L,Whitworth AJ,Lecourtois M,Liévens JC
Thu Jun 01 00:00:00 EDT 2017
Cryptic exon incorporation occurs in Alzheimer's brain lacking TDP-43 inclusion but exhibiting nuclear clearance of TDP-43.
Acta neuropathologica
Sun M,Bell W,LaClair KD,Ling JP,Han H,Kageyama Y,Pletnikova O,Troncoso JC,Wong PC,Chen LL
Thu Jun 01 00:00:00 EDT 2017
Progressive Motor Neuron Pathology and the Role of Astrocytes in a Human Stem Cell Model of VCP-Related ALS.
Cell reports
Hall CE,Yao Z,Choi M,Tyzack GE,Serio A,Luisier R,Harley J,Preza E,Arber C,Crisp SJ,Watson PMD,Kullmann DM,Abramov AY,Wray S,Burley R,Loh SHY,Martins LM,Stevens MM,Luscombe NM,Sibley CR,Lakatos A,Ule J,Gandhi S,Patani R
Tue May 30 00:00:00 EDT 2017
Dioxins and related environmental contaminants increase TDP-43 levels.
Molecular neurodegeneration
Ash PEA,Stanford EA,Al Abdulatif A,Ramirez-Cardenas A,Ballance HI,Boudeau S,Jeh A,Murithi JM,Tripodis Y,Murphy GJ,Sherr DH,Wolozin B
Fri May 05 00:00:00 EDT 2017
Incipient progressive supranuclear palsy is more common than expected and may comprise clinicopathological subtypes: a forensic autopsy series.
Acta neuropathologica
Yoshida K,Hata Y,Kinoshita K,Takashima S,Tanaka K,Nishida N
Mon May 01 00:00:00 EDT 2017
Overexpression of the essential Sis1 chaperone reduces TDP-43 effects on toxicity and proteolysis.
PLoS genetics
Park SK,Hong JY,Arslan F,Kanneganti V,Patel B,Tietsort A,Tank EMH,Li X,Barmada SJ,Liebman SW
Mon May 01 00:00:00 EDT 2017
Clinical Significance of TDP-43 Neuropathology in Amyotrophic Lateral Sclerosis.
Journal of neuropathology and experimental neurology
Cykowski MD,Powell SZ,Peterson LE,Appel JW,Rivera AL,Takei H,Chang E,Appel SH
Mon May 01 00:00:00 EDT 2017
Loss of Ranbp2 in motoneurons causes disruption of nucleocytoplasmic and chemokine signaling, proteostasis of hnRNPH3 and Mmp28, and development of amyotrophic lateral sclerosis-like syndromes.
Disease models & mechanisms
Cho KI,Yoon D,Qiu S,Danziger Z,Grill WM,Wetsel WC,Ferreira PA
Mon May 01 00:00:00 EDT 2017
Heterogeneous ribonuclear protein A3 (hnRNP A3) is present in dipeptide repeat protein containing inclusions in Frontotemporal Lobar Degeneration and Motor Neurone disease associated with expansions in C9orf72 gene.
Acta neuropathologica communications
Davidson YS,Flood L,Robinson AC,Nihei Y,Mori K,Rollinson S,Richardson A,Benson BC,Jones M,Snowden JS,Pickering-Brown S,Haass C,Lashley T,Mann DMA
Fri Apr 21 00:00:00 EDT 2017
Multireceptor fingerprints in progressive supranuclear palsy.
Alzheimer's research & therapy
Chiu WZ,Donker Kaat L,Boon AJW,Kamphorst W,Schleicher A,Zilles K,van Swieten JC,Palomero-Gallagher N
Mon Apr 17 00:00:00 EDT 2017
Survival in the pre-senile dementia frontotemporal lobar degeneration with TDP-43 proteinopathy: effects of genetic, demographic and neuropathological variables.
Folia neuropathologica
Armstrong RA
Thu Apr 13 00:00:00 EDT 2017
Regulatory Role of RNA Chaperone TDP-43 for RNA Misfolding and Repeat-Associated Translation in SCA31.
Neuron
Ishiguro T,Sato N,Ueyama M,Fujikake N,Sellier C,Kanegami A,Tokuda E,Zamiri B,Gall-Duncan T,Mirceta M,Furukawa Y,Yokota T,Wada K,Taylor JP,Pearson CE,Charlet-Berguerand N,Mizusawa H,Nagai Y,Ishikawa K
Wed Apr 05 00:00:00 EDT 2017
Regulatory Role of RNA Chaperone TDP-43 for RNA Misfolding and Repeat-Associated Translation in SCA31.
Neuron
Ishiguro T,Sato N,Ueyama M,Fujikake N,Sellier C,Kanegami A,Tokuda E,Zamiri B,Gall-Duncan T,Mirceta M,Furukawa Y,Yokota T,Wada K,Taylor JP,Pearson CE,Charlet-Berguerand N,Mizusawa H,Nagai Y,Ishikawa K
Wed Apr 05 00:00:00 EDT 2017
VEGF alleviates ALS-CSF induced cytoplasmic accumulations of TDP-43 and FUS/TLS in NSC-34 cells.
Journal of chemical neuroanatomy
Shantanu S,Vijayalakshmi K,Shruthi S,Sagar BKC,Sathyaprabha TN,Nalini A,Raju TR,Alladi PA
Sat Apr 01 00:00:00 EDT 2017
Heat Shock-induced Phosphorylation of TAR DNA-binding Protein 43 (TDP-43) by MAPK/ERK Kinase Regulates TDP-43 Function.
The Journal of biological chemistry
Li W,Reeb AN,Lin B,Subramanian P,Fey EE,Knoverek CR,French RL,Bigio EH,Ayala YM
Fri Mar 24 00:00:00 EDT 2017
Heat Shock-induced Phosphorylation of TAR DNA-binding Protein 43 (TDP-43) by MAPK/ERK Kinase Regulates TDP-43 Function.
The Journal of biological chemistry
Li W,Reeb AN,Lin B,Subramanian P,Fey EE,Knoverek CR,French RL,Bigio EH,Ayala YM
Fri Mar 24 00:00:00 EDT 2017
Amyotrophic lateral sclerosis-linked mutations increase the viscosity of liquid-like TDP-43 RNP granules in neurons.
Proceedings of the National Academy of Sciences of the United States of America
Gopal PP,Nirschl JJ,Klinman E,Holzbaur EL
Tue Mar 21 00:00:00 EDT 2017
Valosin-containing protein (VCP/p97) inhibitors relieve Mitofusin-dependent mitochondrial defects due to VCP disease mutants.
eLife
Zhang T,Mishra P,Hay BA,Chan D,Guo M
Tue Mar 21 00:00:00 EDT 2017
Simple Derivation of Spinal Motor Neurons from ESCs/iPSCs Using Sendai Virus Vectors.
Molecular therapy. Methods & clinical development
Goto K,Imamura K,Komatsu K,Mitani K,Aiba K,Nakatsuji N,Inoue M,Kawata A,Yamashita H,Takahashi R,Inoue H
Fri Mar 17 00:00:00 EDT 2017
C9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten.
Human molecular genetics
Stopford MJ,Higginbottom A,Hautbergue GM,Cooper-Knock J,Mulcahy PJ,De Vos KJ,Renton AE,Pliner H,Calvo A,Chio A,Traynor BJ,Azzouz M,Heath PR,Kirby J,Shaw PJ
Wed Mar 15 00:00:00 EDT 2017
Neuropathological diagnoses and clinical correlates in older adults in Brazil: A cross-sectional study.
PLoS medicine
Suemoto CK,Ferretti-Rebustini RE,Rodriguez RD,Leite RE,Soterio L,Brucki SM,Spera RR,Cippiciani TM,Farfel JM,Chiavegatto Filho A,Naslavsky MS,Zatz M,Pasqualucci CA,Jacob-Filho W,Nitrini R,Grinberg LT
Wed Mar 01 00:00:00 EST 2017
DUX4-induced dsRNA and MYC mRNA stabilization activate apoptotic pathways in human cell models of facioscapulohumeral dystrophy.
PLoS genetics
Shadle SC,Zhong JW,Campbell AE,Conerly ML,Jagannathan S,Wong CJ,Morello TD,van der Maarel SM,Tapscott SJ
Wed Mar 01 00:00:00 EST 2017
Tdp-43 cryptic exons are highly variable between cell types.
Molecular neurodegeneration
Jeong YH,Ling JP,Lin SZ,Donde AN,Braunstein KE,Majounie E,Traynor BJ,LaClair KD,Lloyd TE,Wong PC
Thu Feb 02 00:00:00 EST 2017
Tdp-43 cryptic exons are highly variable between cell types.
Molecular neurodegeneration
Jeong YH,Ling JP,Lin SZ,Donde AN,Braunstein KE,Majounie E,Traynor BJ,LaClair KD,Lloyd TE,Wong PC
Thu Feb 02 00:00:00 EST 2017
TDP-43 protein variants as biomarkers in amyotrophic lateral sclerosis.
BMC neuroscience
Williams SM,Khan G,Harris BT,Ravits J,Sierks MR
Wed Jan 25 00:00:00 EST 2017
Motor-Coordinative and Cognitive Dysfunction Caused by Mutant TDP-43 Could Be Reversed by Inhibiting Its Mitochondrial Localization.
Molecular therapy : the journal of the American Society of Gene Therapy
Wang W,Arakawa H,Wang L,Okolo O,Siedlak SL,Jiang Y,Gao J,Xie F,Petersen RB,Wang X
Wed Jan 04 00:00:00 EST 2017
Motor-Coordinative and Cognitive Dysfunction Caused by Mutant TDP-43 Could Be Reversed by Inhibiting Its Mitochondrial Localization.
Molecular therapy : the journal of the American Society of Gene Therapy
Wang W,Arakawa H,Wang L,Okolo O,Siedlak SL,Jiang Y,Gao J,Xie F,Petersen RB,Wang X
Wed Jan 04 00:00:00 EST 2017
Pathological correlations of [F-18]-AV-1451 imaging in non-alzheimer tauopathies.
Annals of neurology
Marquié M,Normandin MD,Meltzer AC,Siao Tick Chong M,Andrea NV,Antón-Fernández A,Klunk WE,Mathis CA,Ikonomovic MD,Debnath M,Bien EA,Vanderburg CR,Costantino I,Makaretz S,DeVos SL,Oakley DH,Gomperts SN,Growdon JH,Domoto-Reilly K,Lucente D,Dickerson BC,Frosch MP,Hyman BT,Johnson KA,Gómez-Isla T
Sun Jan 01 00:00:00 EST 2017
Pathological TDP-43 changes in Betz cells differ from those in bulbar and spinal α-motoneurons in sporadic amyotrophic lateral sclerosis.
Acta neuropathologica
Braak H,Ludolph AC,Neumann M,Ravits J,Del Tredici K
Sun Jan 01 00:00:00 EST 2017
Imaging specific newly synthesized proteins within cells by fluorescence resonance energy transfer.
Chemical science
Sheng L,Cai L,Liu J,Zhang S,Xu JJ,Zhang X,Chen HY
Sun Jan 01 00:00:00 EST 2017
DYT6 Dystonia: A Neuropathological Study.
Neuro-degenerative diseases
Paudel R,Li A,Hardy J,Bhatia KP,Houlden H,Holton J
Wed Dec 28 00:00:00 EST 2016
Imbalance of mitochondrial dynamics in Drosophila models of amyotrophic lateral sclerosis.
Biochemical and biophysical research communications
Altanbyek V,Cha SJ,Kang GU,Im DS,Lee S,Kim HJ,Kim K
Fri Dec 09 00:00:00 EST 2016
Imbalance of mitochondrial dynamics in Drosophila models of amyotrophic lateral sclerosis.
Biochemical and biophysical research communications
Altanbyek V,Cha SJ,Kang GU,Im DS,Lee S,Kim HJ,Kim K
Fri Dec 09 00:00:00 EST 2016
In vitro prion-like behaviour of TDP-43 in ALS.
Neurobiology of disease
Smethurst P,Newcombe J,Troakes C,Simone R,Chen YR,Patani R,Sidle K
Thu Dec 01 00:00:00 EST 2016
Nuclear bodies reorganize during myogenesis in vitro and are differentially disrupted by expression of FSHD-associated DUX4.
Skeletal muscle
Homma S,Beermann ML,Yu B,Boyce FM,Miller JB
Thu Dec 01 00:00:00 EST 2016
Depletion of TDP-43 decreases fibril and plaque β-amyloid and exacerbates neurodegeneration in an Alzheimer's mouse model.
Acta neuropathologica
LaClair KD,Donde A,Ling JP,Jeong YH,Chhabra R,Martin LJ,Wong PC
Thu Dec 01 00:00:00 EST 2016
Timing and significance of pathological features in C9orf72 expansion-associated frontotemporal dementia.
Brain : a journal of neurology
Vatsavayai SC,Yoon SJ,Gardner RC,Gendron TF,Vargas JN,Trujillo A,Pribadi M,Phillips JJ,Gaus SE,Hixson JD,Garcia PA,Rabinovici GD,Coppola G,Geschwind DH,Petrucelli L,Miller BL,Seeley WW
Thu Dec 01 00:00:00 EST 2016
Familial Amyotrophic Lateral Sclerosis-linked Mutations in Profilin 1 Exacerbate TDP-43-induced Degeneration in the Retina of Drosophila melanogaster through an Increase in the Cytoplasmic Localization of TDP-43.
The Journal of biological chemistry
Matsukawa K,Hashimoto T,Matsumoto T,Ihara R,Chihara T,Miura M,Wakabayashi T,Iwatsubo T
Fri Nov 04 00:00:00 EDT 2016
Familial Amyotrophic Lateral Sclerosis-linked Mutations in Profilin 1 Exacerbate TDP-43-induced Degeneration in the Retina of Drosophila melanogaster through an Increase in the Cytoplasmic Localization of TDP-43.
The Journal of biological chemistry
Matsukawa K,Hashimoto T,Matsumoto T,Ihara R,Chihara T,Miura M,Wakabayashi T,Iwatsubo T
Fri Nov 04 00:00:00 EDT 2016
Unusual Processing Generates SPA LncRNAs that Sequester Multiple RNA Binding Proteins.
Molecular cell
Wu H,Yin QF,Luo Z,Yao RW,Zheng CC,Zhang J,Xiang JF,Yang L,Chen LL
Thu Nov 03 00:00:00 EDT 2016
Unusual Processing Generates SPA LncRNAs that Sequester Multiple RNA Binding Proteins.
Molecular cell
Wu H,Yin QF,Luo Z,Yao RW,Zheng CC,Zhang J,Xiang JF,Yang L,Chen LL
Thu Nov 03 00:00:00 EDT 2016
Tyrosine kinase inhibition reverses TDP-43 effects on synaptic protein expression, astrocytic function and amino acid dis-homeostasis.
Journal of neurochemistry
Heyburn L,Hebron ML,Smith J,Winston C,Bechara J,Li Z,Lonskaya I,Burns MP,Harris BT,Moussa CE
Tue Nov 01 00:00:00 EDT 2016
Tyrosine kinase inhibition reverses TDP-43 effects on synaptic protein expression, astrocytic function and amino acid dis-homeostasis.
Journal of neurochemistry
Heyburn L,Hebron ML,Smith J,Winston C,Bechara J,Li Z,Lonskaya I,Burns MP,Harris BT,Moussa CE
Tue Nov 01 00:00:00 EDT 2016
C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles.
Cell
Lee KH,Zhang P,Kim HJ,Mitrea DM,Sarkar M,Freibaum BD,Cika J,Coughlin M,Messing J,Molliex A,Maxwell BA,Kim NC,Temirov J,Moore J,Kolaitis RM,Shaw TI,Bai B,Peng J,Kriwacki RW,Taylor JP
Thu Oct 20 00:00:00 EDT 2016
Identification of linc-NeD125, a novel long non coding RNA that hosts miR-125b-1 and negatively controls proliferation of human neuroblastoma cells.
RNA biology
Bevilacqua V,Gioia U,Di Carlo V,Tortorelli AF,Colombo T,Bozzoni I,Laneve P,Caffarelli E
Wed Oct 12 00:00:00 EDT 2016
Level of PICALM, a key component of clathrin-mediated endocytosis, is correlated with levels of phosphotau and autophagy-related proteins and is associated with tau inclusions in AD, PSP and Pick disease.
Neurobiology of disease
Ando K,Tomimura K,Sazdovitch V,Suain V,Yilmaz Z,Authelet M,Ndjim M,Vergara C,Belkouch M,Potier MC,Duyckaerts C,Brion JP
Sat Oct 01 00:00:00 EDT 2016
Role of BMP receptor traffic in synaptic growth defects in an ALS model.
Molecular biology of the cell
Deshpande M,Feiger Z,Shilton AK,Luo CC,Silverman E,Rodal AA
Sat Oct 01 00:00:00 EDT 2016
Role of BMP receptor traffic in synaptic growth defects in an ALS model.
Molecular biology of the cell
Deshpande M,Feiger Z,Shilton AK,Luo CC,Silverman E,Rodal AA
Sat Oct 01 00:00:00 EDT 2016
Structural, super-resolution microscopy analysis of paraspeckle nuclear body organization.
The Journal of cell biology
West JA,Mito M,Kurosaka S,Takumi T,Tanegashima C,Chujo T,Yanaka K,Kingston RE,Hirose T,Bond C,Fox A,Nakagawa S
Mon Sep 26 00:00:00 EDT 2016
TDP-43 aggregation mirrors TDP-43 knockdown, affecting the expression levels of a common set of proteins.
Scientific reports
Prpar Mihevc S,Baralle M,Buratti E,Rogelj B
Mon Sep 26 00:00:00 EDT 2016
Shared Molecular Mechanisms in Alzheimer's Disease and Amyotrophic Lateral Sclerosis: Neurofilament-Dependent Transport of sAPP, FUS, TDP-43 and SOD1, with Endoplasmic Reticulum-Like Tubules.
Neuro-degenerative diseases
Muresan V,Ladescu Muresan Z
Tue Sep 20 00:00:00 EDT 2016
Shared Molecular Mechanisms in Alzheimer's Disease and Amyotrophic Lateral Sclerosis: Neurofilament-Dependent Transport of sAPP, FUS, TDP-43 and SOD1, with Endoplasmic Reticulum-Like Tubules.
Neuro-degenerative diseases
Muresan V,Ladescu Muresan Z
Tue Sep 20 00:00:00 EDT 2016
The extreme N-terminus of TDP-43 mediates the cytoplasmic aggregation of TDP-43 and associated toxicity in vivo.
Brain research
Sasaguri H,Chew J,Xu YF,Gendron TF,Garrett A,Lee CW,Jansen-West K,Bauer PO,Perkerson EA,Tong J,Stetler C,Zhang YJ
Thu Sep 15 00:00:00 EDT 2016
Immunoprecipitation and mass spectrometry defines an extensive RBM45 protein-protein interaction network.
Brain research
Li Y,Collins M,An J,Geiser R,Tegeler T,Tsantilas K,Garcia K,Pirrotte P,Bowser R
Thu Sep 15 00:00:00 EDT 2016
Enhancing survival motor neuron expression extends lifespan and attenuates neurodegeneration in mutant TDP-43 mice.
Human molecular genetics
Perera ND,Sheean RK,Crouch PJ,White AR,Horne MK,Turner BJ
Thu Sep 15 00:00:00 EDT 2016
Enhancing survival motor neuron expression extends lifespan and attenuates neurodegeneration in mutant TDP-43 mice.
Human molecular genetics
Perera ND,Sheean RK,Crouch PJ,White AR,Horne MK,Turner BJ
Thu Sep 15 00:00:00 EDT 2016
Mixed tau and TDP-43 pathology in a patient with unclassifiable primary progressive aphasia.
Neurocase
Flanagan EP,Duffy JR,Whitwell JL,Vemuri P,Dickson DW,Josephs KA
Fri Sep 09 00:00:00 EDT 2016
Reduced hnRNPA3 increases C9orf72 repeat RNA levels and dipeptide-repeat protein deposition.
EMBO reports
Mori K,Nihei Y,Arzberger T,Zhou Q,Mackenzie IR,Hermann A,Hanisch F,Kamp F,Nuscher B,Orozco D,Edbauer D,Haass C
Thu Sep 01 00:00:00 EDT 2016
Cytoplasmic aggregates of dynactin in iPSC-derived tyrosine hydroxylase-positive neurons from a patient with Perry syndrome.
Parkinsonism & related disorders
Mishima T,Ishikawa T,Imamura K,Kondo T,Koshiba Y,Takahashi R,Takahashi J,Watanabe A,Fujii N,Tsuboi Y,Inoue H
Thu Sep 01 00:00:00 EDT 2016
Distinct Muscle Biopsy Findings in Genetically Defined Adult-Onset Motor Neuron Disorders.
PloS one
Jokela M,Huovinen S,Raheem O,Lindfors M,Palmio J,Penttilä S,Udd B
Thu Aug 11 00:00:00 EDT 2016
Severe muscle wasting and denervation in mice lacking the RNA-binding protein ZFP106.
Proceedings of the National Academy of Sciences of the United States of America
Anderson DM,Cannavino J,Li H,Anderson KM,Nelson BR,McAnally J,Bezprozvannaya S,Liu Y,Lin W,Liu N,Bassel-Duby R,Olson EN
Tue Aug 02 00:00:00 EDT 2016
Marked Involvement of the Striatal Efferent System in TAR DNA-Binding Protein 43 kDa-Related Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis.
Journal of neuropathology and experimental neurology
Riku Y,Watanabe H,Yoshida M,Mimuro M,Iwasaki Y,Masuda M,Ishigaki S,Katsuno M,Sobue G
Mon Aug 01 00:00:00 EDT 2016
Chronic Traumatic Encephalopathy Presenting as Alzheimer's Disease in a Retired Soccer Player.
Journal of Alzheimer's disease : JAD
Grinberg LT,Anghinah R,Nascimento CF,Amaro E,Leite RP,Martin Mda G,Naslavsky MS,Takada LT,Filho WJ,Pasqualucci CA,Nitrini R
Fri Jul 29 00:00:00 EDT 2016
Lentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteins.
Molecular neurodegeneration
Alves S,Marais T,Biferi MG,Furling D,Marinello M,El Hachimi K,Cartier N,Ruberg M,Stevanin G,Brice A,Barkats M,Sittler A
Thu Jul 28 00:00:00 EDT 2016
Increased cytoplasmic TARDBP mRNA in affected spinal motor neurons in ALS caused by abnormal autoregulation of TDP-43.
Nucleic acids research
Koyama A,Sugai A,Kato T,Ishihara T,Shiga A,Toyoshima Y,Koyama M,Konno T,Hirokawa S,Yokoseki A,Nishizawa M,Kakita A,Takahashi H,Onodera O
Fri Jul 08 00:00:00 EDT 2016
Global identification of hnRNP A1 binding sites for SSO-based splicing modulation.
BMC biology
Bruun GH,Doktor TK,Borch-Jensen J,Masuda A,Krainer AR,Ohno K,Andresen BS
Tue Jul 05 00:00:00 EDT 2016
Distinct and shared functions of ALS-associated proteins TDP-43, FUS and TAF15 revealed by multisystem analyses.
Nature communications
Kapeli K,Pratt GA,Vu AQ,Hutt KR,Martinez FJ,Sundararaman B,Batra R,Freese P,Lambert NJ,Huelga SC,Chun SJ,Liang TY,Chang J,Donohue JP,Shiue L,Zhang J,Zhu H,Cambi F,Kasarskis E,Hoon S,Ares M,Burge CB,Ravits J,Rigo F,Yeo GW
Tue Jul 05 00:00:00 EDT 2016
Argyrophilic Grain Disease: Demographics, Clinical, and Neuropathological Features From a Large Autopsy Study.
Journal of neuropathology and experimental neurology
Rodriguez RD,Suemoto CK,Molina M,Nascimento CF,Leite RE,de Lucena Ferretti-Rebustini RE,Farfel JM,Heinsen H,Nitrini R,Ueda K,Pasqualucci CA,Jacob-Filho W,Yaffe K,Grinberg LT
Fri Jul 01 00:00:00 EDT 2016
The AMPA receptor antagonist perampanel robustly rescues amyotrophic lateral sclerosis (ALS) pathology in sporadic ALS model mice.
Scientific reports
Akamatsu M,Yamashita T,Hirose N,Teramoto S,Kwak S
Tue Jun 28 00:00:00 EDT 2016
TDP-43 Inhibits NF-κB Activity by Blocking p65 Nuclear Translocation.
PloS one
Zhu J,Cynader MS,Jia W
Tue Jun 21 00:00:00 EDT 2016
TDP-43 Inhibits NF-κB Activity by Blocking p65 Nuclear Translocation.
PloS one
Zhu J,Cynader MS,Jia W
Tue Jun 21 00:00:00 EDT 2016
Phenotypic characteristics of Alzheimer patients carrying an ABCA7 mutation.
Neurology
Van den Bossche T,Sleegers K,Cuyvers E,Engelborghs S,Sieben A,De Roeck A,Van Cauwenberghe C,Vermeulen S,Van den Broeck M,Laureys A,Peeters K,Mattheijssens M,Vandenbulcke M,Vandenberghe R,Martin JJ,De Deyn PP,Cras P,Van Broeckhoven C
Tue Jun 07 00:00:00 EDT 2016
Inflammation Induces TDP-43 Mislocalization and Aggregation.
PloS one
Correia AS,Patel P,Dutta K,Julien JP
Tue Jun 07 00:00:00 EDT 2016
Inflammation Induces TDP-43 Mislocalization and Aggregation.
PloS one
Correia AS,Patel P,Dutta K,Julien JP
Tue Jun 07 00:00:00 EDT 2016
Motor neurone disease/amyotrophic lateral sclerosis associated with intermediate-length CAG repeat expansions in Ataxin-2 does not have 1C2-positive polyglutamine inclusions.
Neuropathology and applied neurobiology
Highley JR,Lorente Pons A,Cooper-Knock J,Wharton SB,Ince PG,Shaw PJ,Wood J,Kirby J
Wed Jun 01 00:00:00 EDT 2016
Misregulation of Alternative Splicing in a Mouse Model of Rett Syndrome.
PLoS genetics
Li R,Dong Q,Yuan X,Zeng X,Gao Y,Chiao C,Li H,Zhao X,Keles S,Wang Z,Chang Q
Wed Jun 01 00:00:00 EDT 2016
A novel Drosophila model of TDP-43 proteinopathies: N-terminal sequences combined with the Q/N domain induce protein functional loss and locomotion defects.
Disease models & mechanisms
Langellotti S,Romano V,Romano G,Klima R,Feiguin F,Cragnaz L,Romano M,Baralle FE
Wed Jun 01 00:00:00 EDT 2016
Phosphorylated TDP-43 becomes resistant to cleavage by calpain: A regulatory role for phosphorylation in TDP-43 pathology of ALS/FTLD.
Neuroscience research
Yamashita T,Teramoto S,Kwak S
Wed Jun 01 00:00:00 EDT 2016
Altered localization and functionality of TAR DNA Binding Protein 43 (TDP-43) in niemann- pick disease type C.
Acta neuropathologica communications
Dardis A,Zampieri S,Canterini S,Newell KL,Stuani C,Murrell JR,Ghetti B,Fiorenza MT,Bembi B,Buratti E
Wed May 18 00:00:00 EDT 2016
Proteins with Intrinsically Disordered Domains Are Preferentially Recruited to Polyglutamine Aggregates.
PloS one
Wear MP,Kryndushkin D,O'Meally R,Sonnenberg JL,Cole RN,Shewmaker FP
Wed May 11 00:00:00 EDT 2016
Gene-specific mitochondria dysfunctions in human TARDBP and C9ORF72 fibroblasts.
Acta neuropathologica communications
Onesto E,Colombrita C,Gumina V,Borghi MO,Dusi S,Doretti A,Fagiolari G,Invernizzi F,Moggio M,Tiranti V,Silani V,Ratti A
Thu May 05 00:00:00 EDT 2016
Gene-specific mitochondria dysfunctions in human TARDBP and C9ORF72 fibroblasts.
Acta neuropathologica communications
Onesto E,Colombrita C,Gumina V,Borghi MO,Dusi S,Doretti A,Fagiolari G,Invernizzi F,Moggio M,Tiranti V,Silani V,Ratti A
Thu May 05 00:00:00 EDT 2016
Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs.
Neuron
Jiang J,Zhu Q,Gendron TF,Saberi S,McAlonis-Downes M,Seelman A,Stauffer JE,Jafar-Nejad P,Drenner K,Schulte D,Chun S,Sun S,Ling SC,Myers B,Engelhardt J,Katz M,Baughn M,Platoshyn O,Marsala M,Watt A,Heyser CJ,Ard MC,De Muynck L,Daughrity LM,Swing DA,Tessarollo L,Jung CJ,Delpoux A,Utzschneider DT,Hedrick SM,de Jong PJ,Edbauer D,Van Damme P,Petrucelli L,Shaw CE,Bennett CF,Da Cruz S,Ravits J,Rigo F,Cleveland DW,Lagier-Tourenne C
Wed May 04 00:00:00 EDT 2016
Saccadic Palsy following Cardiac Surgery: Possible Role of Perineuronal Nets.
PloS one
Eggers SD,Horn AK,Roeber S,Härtig W,Nair G,Reich DS,Leigh RJ
Tue May 03 00:00:00 EDT 2016
Poly-dipeptides encoded by the C9ORF72 repeats block global protein translation.
Human molecular genetics
Kanekura K,Yagi T,Cammack AJ,Mahadevan J,Kuroda M,Harms MB,Miller TM,Urano F
Sun May 01 00:00:00 EDT 2016
The heat shock response plays an important role in TDP-43 clearance: evidence for dysfunction in amyotrophic lateral sclerosis.
Brain : a journal of neurology
Chen HJ,Mitchell JC,Novoselov S,Miller J,Nishimura AL,Scotter EL,Vance CA,Cheetham ME,Shaw CE
Sun May 01 00:00:00 EDT 2016
Analysis of RNA metabolism in peripheral WBCs of TDP-43 KI mice identifies novel biomarkers of ALS.
Neuroscience research
Hasegawa M,Hara-Miyauchi C,Ohta H,Sakimura K,Okano H,Okano HJ
Sun May 01 00:00:00 EDT 2016
Analysis of RNA metabolism in peripheral WBCs of TDP-43 KI mice identifies novel biomarkers of ALS.
Neuroscience research
Hasegawa M,Hara-Miyauchi C,Ohta H,Sakimura K,Okano H,Okano HJ
Sun May 01 00:00:00 EDT 2016
Hippocampal Sclerosis but Not Normal Aging or Alzheimer Disease Is Associated With TDP-43 Pathology in the Basal Forebrain of Aged Persons.
Journal of neuropathology and experimental neurology
Cykowski MD,Takei H,Van Eldik LJ,Schmitt FA,Jicha GA,Powell SZ,Nelson PT
Sun May 01 00:00:00 EDT 2016
Targeting TDP-43 phosphorylation by Casein Kinase-1δ inhibitors: a novel strategy for the treatment of frontotemporal dementia.
Molecular neurodegeneration
Alquezar C,Salado IG,de la Encarnación A,Pérez DI,Moreno F,Gil C,de Munain AL,Martínez A,Martín-Requero Á
Sat Apr 30 00:00:00 EDT 2016
Targeting TDP-43 phosphorylation by Casein Kinase-1δ inhibitors: a novel strategy for the treatment of frontotemporal dementia.
Molecular neurodegeneration
Alquezar C,Salado IG,de la Encarnación A,Pérez DI,Moreno F,Gil C,de Munain AL,Martínez A,Martín-Requero Á
Sat Apr 30 00:00:00 EDT 2016
ESCRT-0 dysfunction compromises autophagic degradation of protein aggregates and facilitates ER stress-mediated neurodegeneration via apoptotic and necroptotic pathways.
Scientific reports
Oshima R,Hasegawa T,Tamai K,Sugeno N,Yoshida S,Kobayashi J,Kikuchi A,Baba T,Futatsugi A,Sato I,Satoh K,Takeda A,Aoki M,Tanaka N
Tue Apr 26 00:00:00 EDT 2016
Templated Aggregation of TAR DNA-binding Protein of 43 kDa (TDP-43) by Seeding with TDP-43 Peptide Fibrils.
The Journal of biological chemistry
Shimonaka S,Nonaka T,Suzuki G,Hisanaga S,Hasegawa M
Fri Apr 22 00:00:00 EDT 2016
Templated Aggregation of TAR DNA-binding Protein of 43 kDa (TDP-43) by Seeding with TDP-43 Peptide Fibrils.
The Journal of biological chemistry
Shimonaka S,Nonaka T,Suzuki G,Hisanaga S,Hasegawa M
Fri Apr 22 00:00:00 EDT 2016
ALS-linked protein disulfide isomerase variants cause motor dysfunction.
The EMBO journal
Woehlbier U,Colombo A,Saaranen MJ,Pérez V,Ojeda J,Bustos FJ,Andreu CI,Torres M,Valenzuela V,Medinas DB,Rozas P,Vidal RL,Lopez-Gonzalez R,Salameh J,Fernandez-Collemann S,Muñoz N,Matus S,Armisen R,Sagredo A,Palma K,Irrazabal T,Almeida S,Gonzalez-Perez P,Campero M,Gao FB,Henny P,van Zundert B,Ruddock LW,Concha ML,Henriquez JP,Brown RH,Hetz C
Fri Apr 15 00:00:00 EDT 2016
Multiple sclerosis masquerading as Alzheimer-type dementia: Clinical, radiological and pathological findings.
Multiple sclerosis (Houndmills, Basingstoke, England)
Tobin WO,Popescu BF,Lowe V,Pirko I,Parisi JE,Kantarci K,Fields JA,Bruns MB,Boeve BF,Lucchinetti CF
Fri Apr 01 00:00:00 EDT 2016
G3BP-Caprin1-USP10 complexes mediate stress granule condensation and associate with 40S subunits.
The Journal of cell biology
Kedersha N,Panas MD,Achorn CA,Lyons S,Tisdale S,Hickman T,Thomas M,Lieberman J,McInerney GM,Ivanov P,Anderson P
Mon Mar 28 00:00:00 EDT 2016
C9orf72 is required for proper macrophage and microglial function in mice.
Science (New York, N.Y.)
O'Rourke JG,Bogdanik L,Yáñez A,Lall D,Wolf AJ,Muhammad AK,Ho R,Carmona S,Vit JP,Zarrow J,Kim KJ,Bell S,Harms MB,Miller TM,Dangler CA,Underhill DM,Goodridge HS,Lutz CM,Baloh RH
Fri Mar 18 00:00:00 EDT 2016
Mass spectrometric analysis of accumulated TDP-43 in amyotrophic lateral sclerosis brains.
Scientific reports
Kametani F,Obi T,Shishido T,Akatsu H,Murayama S,Saito Y,Yoshida M,Hasegawa M
Wed Mar 16 00:00:00 EDT 2016
Phosphorylation of TAR DNA-binding Protein of 43 kDa (TDP-43) by Truncated Casein Kinase 1δ Triggers Mislocalization and Accumulation of TDP-43.
The Journal of biological chemistry
Nonaka T,Suzuki G,Tanaka Y,Kametani F,Hirai S,Okado H,Miyashita T,Saitoe M,Akiyama H,Masai H,Hasegawa M
Fri Mar 11 00:00:00 EST 2016
Mechanistic Insights into Hsp104 Potentiation.
The Journal of biological chemistry
Torrente MP,Chuang E,Noll MM,Jackrel ME,Go MS,Shorter J
Fri Mar 04 00:00:00 EST 2016
Loss of VPS13C Function in Autosomal-Recessive Parkinsonism Causes Mitochondrial Dysfunction and Increases PINK1/Parkin-Dependent Mitophagy.
American journal of human genetics
Lesage S,Drouet V,Majounie E,Deramecourt V,Jacoupy M,Nicolas A,Cormier-Dequaire F,Hassoun SM,Pujol C,Ciura S,Erpapazoglou Z,Usenko T,Maurage CA,Sahbatou M,Liebau S,Ding J,Bilgic B,Emre M,Erginel-Unaltuna N,Guven G,Tison F,Tranchant C,Vidailhet M,Corvol JC,Krack P,Leutenegger AL,Nalls MA,Hernandez DG,Heutink P,Gibbs JR,Hardy J,Wood NW,Gasser T,Durr A,Deleuze JF,Tazir M,Destée A,Lohmann E,Kabashi E,Singleton A,Corti O,Brice A
Thu Mar 03 00:00:00 EST 2016
T-Cell-Mediated Inflammatory Myopathies in HIV-Positive Individuals: A Histologic Study of 19 Cases.
Journal of neuropathology and experimental neurology
Hiniker A,Daniels BH,Margeta M
Tue Mar 01 00:00:00 EST 2016
A novel triple repeat mutant tau transgenic model that mimics aspects of pick's disease and fronto-temporal tauopathies.
PloS one
Rockenstein E,Overk CR,Ubhi K,Mante M,Patrick C,Adame A,Bisquert A,Trejo-Morales M,Spencer B,Masliah E
Mon Feb 22 00:00:00 EST 2016
Melatonin enhances arsenic trioxide-induced cell death via sustained upregulation of Redd1 expression in breast cancer cells.
Molecular and cellular endocrinology
Yun SM,Woo SH,Oh ST,Hong SE,Choe TB,Ye SK,Kim EK,Seong MK,Kim HA,Noh WC,Lee JK,Jin HO,Lee YH,Park IC
Mon Feb 15 00:00:00 EST 2016
Diagnostically important muscle pathology in DNAJB6 mutated LGMD1D.
Acta neuropathologica communications
Sandell S,Huovinen S,Palmio J,Raheem O,Lindfors M,Zhao F,Haapasalo H,Udd B
Fri Feb 05 00:00:00 EST 2016
An autopsy case of frontotemporal lobar degeneration with the appearance of fused in sarcoma inclusions (basophilic inclusion body disease) clinically presenting corticobasal syndrome.
Neuropathology : official journal of the Japanese Society of Neuropathology
Matsumoto A,Suzuki H,Fukatsu R,Shimizu H,Suzuki Y,Hisanaga K
Mon Feb 01 00:00:00 EST 2016
Clinical features of TBK1 carriers compared with C9orf72, GRN and non-mutation carriers in a Belgian cohort.
Brain : a journal of neurology
Van Mossevelde S,van der Zee J,Gijselinck I,Engelborghs S,Sieben A,Van Langenhove T,De Bleecker J,Baets J,Vandenbulcke M,Van Laere K,Ceyssens S,Van den Broeck M,Peeters K,Mattheijssens M,Cras P,Vandenberghe R,De Jonghe P,Martin JJ,De Deyn PP,Cruts M,Van Broeckhoven C
Mon Feb 01 00:00:00 EST 2016
Serine 403-phosphorylated p62/SQSTM1 immunoreactivity in inclusions of neurodegenerative diseases.
Neuroscience research
Kurosawa M,Matsumoto G,Sumikura H,Hatsuta H,Murayama S,Sakurai T,Shimogori T,Hattori N,Nukina N
Mon Feb 01 00:00:00 EST 2016
Clinicopathological description of two cases with SQSTM1 gene mutation associated with frontotemporal dementia.
Neuropathology : official journal of the Japanese Society of Neuropathology
Kovacs GG,van der Zee J,Hort J,Kristoferitsch W,Leitha T,Höftberger R,Ströbel T,Van Broeckhoven C,Matej R
Mon Feb 01 00:00:00 EST 2016
TDP-43 loss of function increases TFEB activity and blocks autophagosome-lysosome fusion.
The EMBO journal
Xia Q,Wang H,Hao Z,Fu C,Hu Q,Gao F,Ren H,Chen D,Han J,Ying Z,Wang G
Mon Jan 18 00:00:00 EST 2016
CUL2-mediated clearance of misfolded TDP-43 is paradoxically affected by VHL in oligodendrocytes in ALS.
Scientific reports
Uchida T,Tamaki Y,Ayaki T,Shodai A,Kaji S,Morimura T,Banno Y,Nishitsuji K,Sakashita N,Maki T,Yamashita H,Ito H,Takahashi R,Urushitani M
Mon Jan 11 00:00:00 EST 2016
CUL2-mediated clearance of misfolded TDP-43 is paradoxically affected by VHL in oligodendrocytes in ALS.
Scientific reports
Uchida T,Tamaki Y,Ayaki T,Shodai A,Kaji S,Morimura T,Banno Y,Nishitsuji K,Sakashita N,Maki T,Yamashita H,Ito H,Takahashi R,Urushitani M
Mon Jan 11 00:00:00 EST 2016
MTHFSD and DDX58 are novel RNA-binding proteins abnormally regulated in amyotrophic lateral sclerosis.
Brain : a journal of neurology
MacNair L,Xiao S,Miletic D,Ghani M,Julien JP,Keith J,Zinman L,Rogaeva E,Robertson J
Fri Jan 01 00:00:00 EST 2016
Disparate Mutations Confer Therapeutic Gain of Hsp104 Function.
ACS chemical biology
Jackrel ME,Yee K,Tariq A,Chen AI,Shorter J
Fri Dec 18 00:00:00 EST 2015
Progressive supranuclear palsy in a family with TDP-43 pathology.
Neurocase
Kertesz A,Finger E,Murrell J,Chertkow H,Ang LC,Baker M,Ravenscroft T,Rademakers R,Munoz DG
Thu Dec 17 00:00:00 EST 2015
An age-related reduction of brain TBPH/TDP-43 levels precedes the onset of locomotion defects in a Drosophila ALS model.
Neuroscience
Cragnaz L,Klima R,De Conti L,Romano G,Feiguin F,Buratti E,Baralle M,Baralle FE
Thu Dec 17 00:00:00 EST 2015
C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD.
Neuron
O'Rourke JG,Bogdanik L,Muhammad AKMG,Gendron TF,Kim KJ,Austin A,Cady J,Liu EY,Zarrow J,Grant S,Ho R,Bell S,Carmona S,Simpkinson M,Lall D,Wu K,Daughrity L,Dickson DW,Harms MB,Petrucelli L,Lee EB,Lutz CM,Baloh RH
Wed Dec 02 00:00:00 EST 2015
C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD.
Neuron
O'Rourke JG,Bogdanik L,Muhammad AKMG,Gendron TF,Kim KJ,Austin A,Cady J,Liu EY,Zarrow J,Grant S,Ho R,Bell S,Carmona S,Simpkinson M,Lall D,Wu K,Daughrity L,Dickson DW,Harms MB,Petrucelli L,Lee EB,Lutz CM,Baloh RH
Wed Dec 02 00:00:00 EST 2015
Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice.
Neuron
Peters OM,Cabrera GT,Tran H,Gendron TF,McKeon JE,Metterville J,Weiss A,Wightman N,Salameh J,Kim J,Sun H,Boylan KB,Dickson D,Kennedy Z,Lin Z,Zhang YJ,Daughrity L,Jung C,Gao FB,Sapp PC,Horvitz HR,Bosco DA,Brown SP,de Jong P,Petrucelli L,Mueller C,Brown RH
Wed Dec 02 00:00:00 EST 2015
Pathological features of preclinical or early clinical stages of corticobasal degeneration: a comparison with advanced cases.
Neuropathology and applied neurobiology
Nishida N,Yoshida K,Hata Y,Arai Y,Kinoshita K
Tue Dec 01 00:00:00 EST 2015
Quantitative analysis and clinico-pathological correlations of different dipeptide repeat protein pathologies in C9ORF72 mutation carriers.
Acta neuropathologica
Mackenzie IR,Frick P,Grässer FA,Gendron TF,Petrucelli L,Cashman NR,Edbauer D,Kremmer E,Prudlo J,Troost D,Neumann M
Tue Dec 01 00:00:00 EST 2015
Myofibrillar disruption and RNA-binding protein aggregation in a mouse model of limb-girdle muscular dystrophy 1D.
Human molecular genetics
Bengoechea R,Pittman SK,Tuck EP,True HL,Weihl CC
Tue Dec 01 00:00:00 EST 2015
From transcriptomic to protein level changes in TDP-43 and FUS loss-of-function cell models.
Biochimica et biophysica acta
Colombrita C,Onesto E,Buratti E,de la Grange P,Gumina V,Baralle FE,Silani V,Ratti A
Tue Dec 01 00:00:00 EST 2015
From transcriptomic to protein level changes in TDP-43 and FUS loss-of-function cell models.
Biochimica et biophysica acta
Colombrita C,Onesto E,Buratti E,de la Grange P,Gumina V,Baralle FE,Silani V,Ratti A
Tue Dec 01 00:00:00 EST 2015
TDP-43 is intercellularly transmitted across axon terminals.
The Journal of cell biology
Feiler MS,Strobel B,Freischmidt A,Helferich AM,Kappel J,Brewer BM,Li D,Thal DR,Walther P,Ludolph AC,Danzer KM,Weishaupt JH
Mon Nov 23 00:00:00 EST 2015
TDP-43 is intercellularly transmitted across axon terminals.
The Journal of cell biology
Feiler MS,Strobel B,Freischmidt A,Helferich AM,Kappel J,Brewer BM,Li D,Thal DR,Walther P,Ludolph AC,Danzer KM,Weishaupt JH
Mon Nov 23 00:00:00 EST 2015
Amyotrophic lateral sclerosis-associated mutant profilin 1 increases dendritic arborisation and spine formation in primary hippocampal neurons.
Neuroscience letters
Brettle M,Suchowerska AK,Chua SW,Ittner LM,Fath T
Mon Nov 16 00:00:00 EST 2015
Dysregulated miRNA biogenesis downstream of cellular stress and ALS-causing mutations: a new mechanism for ALS.
The EMBO journal
Emde A,Eitan C,Liou LL,Libby RT,Rivkin N,Magen I,Reichenstein I,Oppenheim H,Eilam R,Silvestroni A,Alajajian B,Ben-Dov IZ,Aebischer J,Savidor A,Levin Y,Sons R,Hammond SM,Ravits JM,Möller T,Hornstein E
Tue Nov 03 00:00:00 EST 2015
Early maturation and distinct tau pathology in induced pluripotent stem cell-derived neurons from patients with MAPT mutations.
Brain : a journal of neurology
Iovino M,Agathou S,González-Rueda A,Del Castillo Velasco-Herrera M,Borroni B,Alberici A,Lynch T,O'Dowd S,Geti I,Gaffney D,Vallier L,Paulsen O,Káradóttir RT,Spillantini MG
Sun Nov 01 00:00:00 EDT 2015
Validating novel tau positron emission tomography tracer [F-18]-AV-1451 (T807) on postmortem brain tissue.
Annals of neurology
Marquié M,Normandin MD,Vanderburg CR,Costantino IM,Bien EA,Rycyna LG,Klunk WE,Mathis CA,Ikonomovic MD,Debnath ML,Vasdev N,Dickerson BC,Gomperts SN,Growdon JH,Johnson KA,Frosch MP,Hyman BT,Gómez-Isla T
Sun Nov 01 00:00:00 EDT 2015
Stress Granules Modulate SYK to Cause Microglial Cell Dysfunction in Alzheimer's Disease.
EBioMedicine
Ghosh S,Geahlen RL
Sun Nov 01 00:00:00 EDT 2015
Short-term suppression of A315T mutant human TDP-43 expression improves functional deficits in a novel inducible transgenic mouse model of FTLD-TDP and ALS.
Acta neuropathologica
Ke YD,van Hummel A,Stevens CH,Gladbach A,Ippati S,Bi M,Lee WS,Krüger S,van der Hoven J,Volkerling A,Bongers A,Halliday G,Haass NK,Kiernan M,Delerue F,Ittner LM
Sun Nov 01 00:00:00 EDT 2015
Short-term suppression of A315T mutant human TDP-43 expression improves functional deficits in a novel inducible transgenic mouse model of FTLD-TDP and ALS.
Acta neuropathologica
Ke YD,van Hummel A,Stevens CH,Gladbach A,Ippati S,Bi M,Lee WS,Krüger S,van der Hoven J,Volkerling A,Bongers A,Halliday G,Haass NK,Kiernan M,Delerue F,Ittner LM
Sun Nov 01 00:00:00 EDT 2015
Ubiquilin-2 drives NF-κB activity and cytosolic TDP-43 aggregation in neuronal cells.
Molecular brain
Picher-Martel V,Dutta K,Phaneuf D,Sobue G,Julien JP
Sat Oct 31 00:00:00 EDT 2015
Ubiquilin-2 drives NF-κB activity and cytosolic TDP-43 aggregation in neuronal cells.
Molecular brain
Picher-Martel V,Dutta K,Phaneuf D,Sobue G,Julien JP
Sat Oct 31 00:00:00 EDT 2015
Phenotype of transgenic mice carrying a very low copy number of the mutant human G93A superoxide dismutase-1 gene associated with amyotrophic lateral sclerosis.
PloS one
Deitch JS,Alexander GM,Bensinger A,Yang S,Jiang JT,Heiman-Patterson TD
Mon Oct 19 00:00:00 EDT 2015
AAV9 supports wide-scale transduction of the CNS and TDP-43 disease modeling in adult rats.
Molecular therapy. Methods & clinical development
Jackson KL,Dayton RD,Klein RL
Thu Oct 08 00:00:00 EDT 2015
PABPN1 suppresses TDP-43 toxicity in ALS disease models.
Human molecular genetics
Chou CC,Alexeeva OM,Yamada S,Pribadi A,Zhang Y,Mo B,Williams KR,Zarnescu DC,Rossoll W
Tue Sep 15 00:00:00 EDT 2015
PABPN1 suppresses TDP-43 toxicity in ALS disease models.
Human molecular genetics
Chou CC,Alexeeva OM,Yamada S,Pribadi A,Zhang Y,Mo B,Williams KR,Zarnescu DC,Rossoll W
Tue Sep 15 00:00:00 EDT 2015
C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits.
Annals of neurology
Koppers M,Blokhuis AM,Westeneng HJ,Terpstra ML,Zundel CA,Vieira de Sá R,Schellevis RD,Waite AJ,Blake DJ,Veldink JH,van den Berg LH,Pasterkamp RJ
Tue Sep 01 00:00:00 EDT 2015
Spinocerebellar ataxia type 36 exists in diverse populations and can be caused by a short hexanucleotide GGCCTG repeat expansion.
Journal of neurology, neurosurgery, and psychiatry
Obayashi M,Stevanin G,Synofzik M,Monin ML,Duyckaerts C,Sato N,Streichenberger N,Vighetto A,Desestret V,Tesson C,Wichmann HE,Illig T,Huttenlocher J,Kita Y,Izumi Y,Mizusawa H,Schöls L,Klopstock T,Brice A,Ishikawa K,Dürr A
Tue Sep 01 00:00:00 EDT 2015
SQSTM1 splice site mutation in distal myopathy with rimmed vacuoles.
Neurology
Bucelli RC,Arhzaouy K,Pestronk A,Pittman SK,Rojas L,Sue CM,Evilä A,Hackman P,Udd B,Harms MB,Weihl CC
Tue Aug 25 00:00:00 EDT 2015
4-Hydroxynonenal induces persistent insolubilization of TDP-43 and alters its intracellular localization.
Biochemical and biophysical research communications
Kabuta C,Kono K,Wada K,Kabuta T
Mon Aug 24 00:00:00 EDT 2015
Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions.
Scientific reports
Farrawell NE,Lambert-Smith IA,Warraich ST,Blair IP,Saunders DN,Hatters DM,Yerbury JJ
Fri Aug 21 00:00:00 EDT 2015
Common pathobiochemical hallmarks of progranulin-associated frontotemporal lobar degeneration and neuronal ceroid lipofuscinosis.
Acta neuropathologica
Götzl JK,Mori K,Damme M,Fellerer K,Tahirovic S,Kleinberger G,Janssens J,van der Zee J,Lang CM,Kremmer E,Martin JJ,Engelborghs S,Kretzschmar HA,Arzberger T,Van Broeckhoven C,Haass C,Capell A
Thu Aug 20 00:00:00 EDT 2015
Age-Dependent TDP-43-Mediated Motor Neuron Degeneration Requires GSK3, hat-trick, and xmas-2.
Current biology : CB
Sreedharan J,Neukomm LJ,Brown RH,Freeman MR
Mon Aug 17 00:00:00 EDT 2015
A phosphomimetic mutant TDP-43 (S409/410E) induces Drosha instability and cytotoxicity in Neuro 2A cells.
Biochemical and biophysical research communications
Kim KY,Lee HW,Shim YM,Mook-Jung I,Jeon GS,Sung JJ
Fri Aug 14 00:00:00 EDT 2015
An optimized InCell Western screening technique identifies hexachlorophene as a novel potent TDP43 targeting drug.
Journal of biotechnology
Narayan M,Peralta DA,Gibson C,Zitnyar A,Jinwal UK
Mon Aug 10 00:00:00 EDT 2015
TDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD.
Science (New York, N.Y.)
Ling JP,Pletnikova O,Troncoso JC,Wong PC
Fri Aug 07 00:00:00 EDT 2015
TDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD.
Science (New York, N.Y.)
Ling JP,Pletnikova O,Troncoso JC,Wong PC
Fri Aug 07 00:00:00 EDT 2015
Traumatic brain injury causes frontotemporal dementia and TDP-43 proteolysis.
Neuroscience
Wang HK,Lee YC,Huang CY,Liliang PC,Lu K,Chen HJ,Li YC,Tsai KJ
Thu Aug 06 00:00:00 EDT 2015
Expanding the spectrum of neuronal pathology in multiple system atrophy.
Brain : a journal of neurology
Cykowski MD,Coon EA,Powell SZ,Jenkins SM,Benarroch EE,Low PA,Schmeichel AM,Parisi JE
Sat Aug 01 00:00:00 EDT 2015
Identification of transactivation-responsive DNA-binding protein 43 (TARDBP43; TDP-43) as a novel factor for TNF-α expression upon lipopolysaccharide stimulation in human monocytes.
Journal of periodontal research
Murata H,Hattori T,Maeda H,Takashiba S,Takigawa M,Kido J,Nagata T
Sat Aug 01 00:00:00 EDT 2015
Identification of transactivation-responsive DNA-binding protein 43 (TARDBP43; TDP-43) as a novel factor for TNF-α expression upon lipopolysaccharide stimulation in human monocytes.
Journal of periodontal research
Murata H,Hattori T,Maeda H,Takashiba S,Takigawa M,Kido J,Nagata T
Sat Aug 01 00:00:00 EDT 2015
Identification of transactivation-responsive DNA-binding protein 43 (TARDBP43; TDP-43) as a novel factor for TNF-α expression upon lipopolysaccharide stimulation in human monocytes.
Journal of periodontal research
Murata H,Hattori T,Maeda H,Takashiba S,Takigawa M,Kido J,Nagata T
Sat Aug 01 00:00:00 EDT 2015
Evaluation of Skin Fibroblasts from Amyotrophic Lateral Sclerosis Patients for the Rapid Study of Pathological Features.
Neurotoxicity research
Yang S,Zhang KY,Kariawasam R,Bax M,Fifita JA,Ooi L,Yerbury JJ,Nicholson GA,Blair IP
Sat Aug 01 00:00:00 EDT 2015
R47H Variant of TREM2 Associated With Alzheimer Disease in a Large Late-Onset Family: Clinical, Genetic, and Neuropathological Study.
JAMA neurology
Korvatska O,Leverenz JB,Jayadev S,McMillan P,Kurtz I,Guo X,Rumbaugh M,Matsushita M,Girirajan S,Dorschner MO,Kiianitsa K,Yu CE,Brkanac Z,Garden GA,Raskind WH,Bird TD
Sat Aug 01 00:00:00 EDT 2015
G1/S Cell Cycle Checkpoint Dysfunction in Lymphoblasts from Sporadic Parkinson's Disease Patients.
Molecular neurobiology
Esteras N,Alquézar C,Bartolomé F,de la Encarnación A,Bermejo-Pareja F,Molina JA,Martín-Requero Á
Sat Aug 01 00:00:00 EDT 2015
G1/S Cell Cycle Checkpoint Dysfunction in Lymphoblasts from Sporadic Parkinson's Disease Patients.
Molecular neurobiology
Esteras N,Alquézar C,Bartolomé F,de la Encarnación A,Bermejo-Pareja F,Molina JA,Martín-Requero Á
Sat Aug 01 00:00:00 EDT 2015
Spread of pathology in amyotrophic lateral sclerosis: assessment of phosphorylated TDP-43 along axonal pathways.
Acta neuropathologica communications
Fatima M,Tan R,Halliday GM,Kril JJ
Tue Jul 28 00:00:00 EDT 2015
Complete loss of the DNAJB6 G/F domain and novel missense mutations cause distal-onset DNAJB6 myopathy.
Acta neuropathologica communications
Ruggieri A,Brancati F,Zanotti S,Maggi L,Pasanisi MB,Saredi S,Terracciano C,Antozzi C,D Apice MR,Sangiuolo F,Novelli G,Marshall CR,Scherer SW,Morandi L,Federici L,Massa R,Mora M,Minassian BA
Sat Jul 25 00:00:00 EDT 2015
Neuropathologic analysis of Tyr69His TTR variant meningovascular amyloidosis with dementia.
Acta neuropathologica communications
Ziskin JL,Greicius MD,Zhu W,Okumu AN,Adams CM,Plowey ED
Fri Jul 10 00:00:00 EDT 2015
Antisense RNA foci in the motor neurons of C9ORF72-ALS patients are associated with TDP-43 proteinopathy.
Acta neuropathologica
Cooper-Knock J,Higginbottom A,Stopford MJ,Highley JR,Ince PG,Wharton SB,Pickering-Brown S,Kirby J,Hautbergue GM,Shaw PJ
Wed Jul 01 00:00:00 EDT 2015
Low molecular weight species of TDP-43 generated by abnormal splicing form inclusions in amyotrophic lateral sclerosis and result in motor neuron death.
Acta neuropathologica
Xiao S,Sanelli T,Chiang H,Sun Y,Chakrabartty A,Keith J,Rogaeva E,Zinman L,Robertson J
Wed Jul 01 00:00:00 EDT 2015
Low molecular weight species of TDP-43 generated by abnormal splicing form inclusions in amyotrophic lateral sclerosis and result in motor neuron death.
Acta neuropathologica
Xiao S,Sanelli T,Chiang H,Sun Y,Chakrabartty A,Keith J,Rogaeva E,Zinman L,Robertson J
Wed Jul 01 00:00:00 EDT 2015
Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS.
Acta neuropathologica communications
Mitchell JC,Constable R,So E,Vance C,Scotter E,Glover L,Hortobagyi T,Arnold ES,Ling SC,McAlonis M,Da Cruz S,Polymenidou M,Tessarolo L,Cleveland DW,Shaw CE
Thu Jun 25 00:00:00 EDT 2015
Oxr1 improves pathogenic cellular features of ALS-associated FUS and TDP-43 mutations.
Human molecular genetics
Finelli MJ,Liu KX,Wu Y,Oliver PL,Davies KE
Mon Jun 15 00:00:00 EDT 2015
Miro1 deficiency in amyotrophic lateral sclerosis.
Frontiers in aging neuroscience
Zhang F,Wang W,Siedlak SL,Liu Y,Liu J,Jiang K,Perry G,Zhu X,Wang X
Mon Jun 15 00:00:00 EDT 2015
Neurodegeneration in C. elegans models of ALS requires TIR-1/Sarm1 immune pathway activation in neurons.
Nature communications
Vérièpe J,Fossouo L,Parker JA
Wed Jun 10 00:00:00 EDT 2015
Neurodegeneration. C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits.
Science (New York, N.Y.)
Chew J,Gendron TF,Prudencio M,Sasaguri H,Zhang YJ,Castanedes-Casey M,Lee CW,Jansen-West K,Kurti A,Murray ME,Bieniek KF,Bauer PO,Whitelaw EC,Rousseau L,Stankowski JN,Stetler C,Daughrity LM,Perkerson EA,Desaro P,Johnston A,Overstreet K,Edbauer D,Rademakers R,Boylan KB,Dickson DW,Fryer JD,Petrucelli L
Fri Jun 05 00:00:00 EDT 2015
Minimal change multiple system atrophy: an aggressive variant?
Movement disorders : official journal of the Movement Disorder Society
Ling H,Asi YT,Petrovic IN,Ahmed Z,Prashanth LK,Hazrati LN,Nishizawa M,Ozawa T,Lang A,Lees AJ,Revesz T,Holton JL
Mon Jun 01 00:00:00 EDT 2015
Mutant TDP-43 deregulates AMPK activation by PP2A in ALS models.
PloS one
Perera ND,Sheean RK,Scott JW,Kemp BE,Horne MK,Turner BJ
Mon Jun 01 00:00:00 EDT 2015
Mutant TDP-43 deregulates AMPK activation by PP2A in ALS models.
PloS one
Perera ND,Sheean RK,Scott JW,Kemp BE,Horne MK,Turner BJ
Mon Jun 01 00:00:00 EDT 2015
Gene targeting of mouse Tardbp negatively affects Masp2 expression.
PloS one
Dib S,Xiao S,Miletic D,Robertson J
Mon Jun 01 00:00:00 EDT 2015
Hippocampal sclerosis in the parkinsonism-dementia complex of Guam: quantitative examination of neurons, neurofibrillary tangles, and TDP-43 immunoreactivity in CA1.
Neuropathology : official journal of the Japanese Society of Neuropathology
Oyanagi K,Yamazaki M,Hashimoto T,Asakawa M,Wakabayashi K,Takahashi H
Mon Jun 01 00:00:00 EDT 2015
Proteomic analyses reveal that loss of TDP-43 affects RNA processing and intracellular transport.
Neuroscience
Štalekar M,Yin X,Rebolj K,Darovic S,Troakes C,Mayr M,Shaw CE,Rogelj B
Thu May 07 00:00:00 EDT 2015
Proteomic analyses reveal that loss of TDP-43 affects RNA processing and intracellular transport.
Neuroscience
Štalekar M,Yin X,Rebolj K,Darovic S,Troakes C,Mayr M,Shaw CE,Rogelj B
Thu May 07 00:00:00 EDT 2015
Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia.
Nature neuroscience
Freischmidt A,Wieland T,Richter B,Ruf W,Schaeffer V,Müller K,Marroquin N,Nordin F,Hübers A,Weydt P,Pinto S,Press R,Millecamps S,Molko N,Bernard E,Desnuelle C,Soriani MH,Dorst J,Graf E,Nordström U,Feiler MS,Putz S,Boeckers TM,Meyer T,Winkler AS,Winkelman J,de Carvalho M,Thal DR,Otto M,Brännström T,Volk AE,Kursula P,Danzer KM,Lichtner P,Dikic I,Meitinger T,Ludolph AC,Strom TM,Andersen PM,Weishaupt JH
Fri May 01 00:00:00 EDT 2015
Analysis of β-N-methylamino-L-alanine (L-BMAA) neurotoxicity in rat cerebellum.
Neurotoxicology
Muñoz-Sáez E,de Munck García E,Arahuetes Portero RM,Martínez A,Solas Alados MT,Miguel BG
Fri May 01 00:00:00 EDT 2015
Analysis of β-N-methylamino-L-alanine (L-BMAA) neurotoxicity in rat cerebellum.
Neurotoxicology
Muñoz-Sáez E,de Munck García E,Arahuetes Portero RM,Martínez A,Solas Alados MT,Miguel BG
Fri May 01 00:00:00 EDT 2015
Neuropathologic features of suicide victims who presented with acute poststroke depression: significance of association with neurodegenerative disorders.
Journal of neuropathology and experimental neurology
Nishida N,Hata Y,Yoshida K,Kinoshita K
Fri May 01 00:00:00 EDT 2015
Nuclear accumulation of mRNAs underlies G4C2-repeat-induced translational repression in a cellular model of C9orf72 ALS.
Journal of cell science
Rossi S,Serrano A,Gerbino V,Giorgi A,Di Francesco L,Nencini M,Bozzo F,Schininà ME,Bagni C,Cestra G,Carrì MT,Achsel T,Cozzolino M
Fri May 01 00:00:00 EDT 2015
A Peruvian family with a novel PARK2 mutation: Clinical and pathological characteristics.
Parkinsonism & related disorders
Cornejo-Olivas MR,Torres L,Mata IF,Mazzetti P,Rivas D,Cosentino C,Inca-Martinez M,Cuba JM,Zabetian CP,Leverenz JB
Fri May 01 00:00:00 EDT 2015
Primary fibroblasts cultures reveal TDP-43 abnormalities in amyotrophic lateral sclerosis patients with and without SOD1 mutations.
Neurobiology of aging
Sabatelli M,Zollino M,Conte A,Del Grande A,Marangi G,Lucchini M,Mirabella M,Romano A,Piacentini R,Bisogni G,Lattante S,Luigetti M,Rossini PM,Moncada A
Fri May 01 00:00:00 EDT 2015
Nucleolar stress and impaired stress granule formation contribute to C9orf72 RAN translation-induced cytotoxicity.
Human molecular genetics
Tao Z,Wang H,Xia Q,Li K,Li K,Jiang X,Xu G,Wang G,Ying Z
Fri May 01 00:00:00 EDT 2015
FUS/TLS deficiency causes behavioral and pathological abnormalities distinct from amyotrophic lateral sclerosis.
Acta neuropathologica communications
Kino Y,Washizu C,Kurosawa M,Yamada M,Miyazaki H,Akagi T,Hashikawa T,Doi H,Takumi T,Hicks GG,Hattori N,Shimogori T,Nukina N
Sat Apr 25 00:00:00 EDT 2015
G3BP1 promotes stress-induced RNA granule interactions to preserve polyadenylated mRNA.
The Journal of cell biology
Aulas A,Caron G,Gkogkas CG,Mohamed NV,Destroismaisons L,Sonenberg N,Leclerc N,Parker JA,Vande Velde C
Mon Apr 13 00:00:00 EDT 2015
G3BP1 promotes stress-induced RNA granule interactions to preserve polyadenylated mRNA.
The Journal of cell biology
Aulas A,Caron G,Gkogkas CG,Mohamed NV,Destroismaisons L,Sonenberg N,Leclerc N,Parker JA,Vande Velde C
Mon Apr 13 00:00:00 EDT 2015
A novel mutation P112H in the TARDBP gene associated with frontotemporal lobar degeneration without motor neuron disease and abundant neuritic amyloid plaques.
Acta neuropathologica communications
Moreno F,Rabinovici GD,Karydas A,Miller Z,Hsu SC,Legati A,Fong J,Schonhaut D,Esselmann H,Watson C,Stephens ML,Kramer J,Wiltfang J,Seeley WW,Miller BL,Coppola G,Grinberg LT
Fri Apr 03 00:00:00 EDT 2015
TDP-43 as a possible biomarker for frontotemporal lobar degeneration: a systematic review of existing antibodies.
Acta neuropathologica communications
Goossens J,Vanmechelen E,Trojanowski JQ,Lee VM,Van Broeckhoven C,van der Zee J,Engelborghs S
Wed Apr 01 00:00:00 EDT 2015
Initial gene vector dosing for studying symptomatology of amyotrophic lateral sclerosis in non-human primates.
Journal of medical primatology
Jackson KL,Dayton RD,Fisher-Perkins JM,Didier PJ,Baker KC,Weimer M,Gutierrez A,Cain CD,Mathis JM,Gitcho MA,Bunnell BA,Klein RL
Wed Apr 01 00:00:00 EDT 2015
Initial gene vector dosing for studying symptomatology of amyotrophic lateral sclerosis in non-human primates.
Journal of medical primatology
Jackson KL,Dayton RD,Fisher-Perkins JM,Didier PJ,Baker KC,Weimer M,Gutierrez A,Cain CD,Mathis JM,Gitcho MA,Bunnell BA,Klein RL
Wed Apr 01 00:00:00 EDT 2015
Peptidylprolyl isomerase A governs TARDBP function and assembly in heterogeneous nuclear ribonucleoprotein complexes.
Brain : a journal of neurology
Lauranzano E,Pozzi S,Pasetto L,Stucchi R,Massignan T,Paolella K,Mombrini M,Nardo G,Lunetta C,Corbo M,Mora G,Bendotti C,Bonetto V
Wed Apr 01 00:00:00 EDT 2015
Characterization of the dipeptide repeat protein in the molecular pathogenesis of c9FTD/ALS.
Human molecular genetics
Yamakawa M,Ito D,Honda T,Kubo K,Noda M,Nakajima K,Suzuki N
Sun Mar 15 00:00:00 EDT 2015
Characterization of the dipeptide repeat protein in the molecular pathogenesis of c9FTD/ALS.
Human molecular genetics
Yamakawa M,Ito D,Honda T,Kubo K,Noda M,Nakajima K,Suzuki N
Sun Mar 15 00:00:00 EDT 2015
Phosphorylation of hnRNP K by cyclin-dependent kinase 2 controls cytosolic accumulation of TDP-43.
Human molecular genetics
Moujalled D,James JL,Yang S,Zhang K,Duncan C,Moujalled DM,Parker SJ,Caragounis A,Lidgerwood G,Turner BJ,Atkin JD,Grubman A,Liddell JR,Proepper C,Boeckers TM,Kanninen KM,Blair I,Crouch PJ,White AR
Sun Mar 15 00:00:00 EDT 2015
Phosphorylation of hnRNP K by cyclin-dependent kinase 2 controls cytosolic accumulation of TDP-43.
Human molecular genetics
Moujalled D,James JL,Yang S,Zhang K,Duncan C,Moujalled DM,Parker SJ,Caragounis A,Lidgerwood G,Turner BJ,Atkin JD,Grubman A,Liddell JR,Proepper C,Boeckers TM,Kanninen KM,Blair I,Crouch PJ,White AR
Sun Mar 15 00:00:00 EDT 2015
Phosphorylation of hnRNP K by cyclin-dependent kinase 2 controls cytosolic accumulation of TDP-43.
Human molecular genetics
Moujalled D,James JL,Yang S,Zhang K,Duncan C,Moujalled DM,Parker SJ,Caragounis A,Lidgerwood G,Turner BJ,Atkin JD,Grubman A,Liddell JR,Proepper C,Boeckers TM,Kanninen KM,Blair I,Crouch PJ,White AR
Sun Mar 15 00:00:00 EDT 2015
Nuclear TDP-43 causes neuronal toxicity by escaping from the inhibitory regulation by hnRNPs.
Human molecular genetics
Suzuki H,Shibagaki Y,Hattori S,Matsuoka M
Sun Mar 15 00:00:00 EDT 2015
Nuclear TDP-43 causes neuronal toxicity by escaping from the inhibitory regulation by hnRNPs.
Human molecular genetics
Suzuki H,Shibagaki Y,Hattori S,Matsuoka M
Sun Mar 15 00:00:00 EDT 2015
Defining neurodegeneration on Guam by targeted genomic sequencing.
Annals of neurology
Steele JC,Guella I,Szu-Tu C,Lin MK,Thompson C,Evans DM,Sherman HE,Vilariño-Güell C,Gwinn K,Morris H,Dickson DW,Farrer MJ
Sun Mar 01 00:00:00 EST 2015
Increasing progranulin levels and blockade of the ERK1/2 pathway: upstream and downstream strategies for the treatment of progranulin deficient frontotemporal dementia.
European neuropsychopharmacology : the journal of the European College of Neuropsychopharmacology
Alquezar C,Esteras N,de la Encarnación A,Moreno F,López de Munain A,Martín-Requero Á
Sun Mar 01 00:00:00 EST 2015
Pathogenic Ubqln2 gains toxic properties to induce neuron death.
Acta neuropathologica
Wu Q,Liu M,Huang C,Liu X,Huang B,Li N,Zhou H,Xia XG
Sun Mar 01 00:00:00 EST 2015
TDP-43 modification in the hSOD1(G93A) amyotrophic lateral sclerosis mouse model.
Neurological research
Cai M,Lee KW,Choi SM,Yang EJ
Sun Mar 01 00:00:00 EST 2015
TDP-43 modification in the hSOD1(G93A) amyotrophic lateral sclerosis mouse model.
Neurological research
Cai M,Lee KW,Choi SM,Yang EJ
Sun Mar 01 00:00:00 EST 2015
TARDBP pathogenic mutations increase cytoplasmic translocation of TDP-43 and cause reduction of endoplasmic reticulum Ca²⁺ signaling in motor neurons.
Neurobiology of disease
Mutihac R,Alegre-Abarrategui J,Gordon D,Farrimond L,Yamasaki-Mann M,Talbot K,Wade-Martins R
Sun Mar 01 00:00:00 EST 2015
Novel atomic force microscopy based biopanning for isolation of morphology specific reagents against TDP-43 variants in amyotrophic lateral sclerosis.
Journal of visualized experiments : JoVE
Williams SM,Venkataraman L,Tian H,Khan G,Harris BT,Sierks MR
Thu Feb 12 00:00:00 EST 2015
Mutant TDP-43 deregulates AMPK activation by PP2A in ALS models.
PloS one
Perera ND,Sheean RK,Scott JW,Kemp BE,Horne MK,Turner BJ
Mon Feb 09 00:00:00 EST 2015
Casein kinase II induced polymerization of soluble TDP-43 into filaments is inhibited by heat shock proteins.
PloS one
Carlomagno Y,Zhang Y,Davis M,Lin WL,Cook C,Dunmore J,Tay W,Menkosky K,Cao X,Petrucelli L,Deture M
Mon Feb 09 00:00:00 EST 2015
Th1 response and systemic treg deficiency in inclusion body myositis.
PloS one
Allenbach Y,Chaara W,Rosenzwajg M,Six A,Prevel N,Mingozzi F,Wanschitz J,Musset L,Charuel JL,Eymard B,Salomon B,Duyckaerts C,Maisonobe T,Dubourg O,Herson S,Klatzmann D,Benveniste O
Mon Feb 09 00:00:00 EST 2015
Opposing roles of p38 and JNK in a Drosophila model of TDP-43 proteinopathy reveal oxidative stress and innate immunity as pathogenic components of neurodegeneration.
Human molecular genetics
Zhan L,Xie Q,Tibbetts RS
Sun Feb 01 00:00:00 EST 2015
Changes in TDP-43 expression in development, aging, and in the neurofilament light protein knockout mouse.
Neurobiology of aging
Liu Y,Atkinson RA,Fernandez-Martos CM,Kirkcaldie MT,Cui H,Vickers JC,King AE
Sun Feb 01 00:00:00 EST 2015
Changes in TDP-43 expression in development, aging, and in the neurofilament light protein knockout mouse.
Neurobiology of aging
Liu Y,Atkinson RA,Fernandez-Martos CM,Kirkcaldie MT,Cui H,Vickers JC,King AE
Sun Feb 01 00:00:00 EST 2015
Expression of FSHD-related DUX4-FL alters proteostasis and induces TDP-43 aggregation.
Annals of clinical and translational neurology
Homma S,Beermann ML,Boyce FM,Miller JB
Sun Feb 01 00:00:00 EST 2015
The cleavage pattern of TDP-43 determines its rate of clearance and cytotoxicity.
Nature communications
Li Q,Yokoshi M,Okada H,Kawahara Y
Thu Jan 29 00:00:00 EST 2015
The cleavage pattern of TDP-43 determines its rate of clearance and cytotoxicity.
Nature communications
Li Q,Yokoshi M,Okada H,Kawahara Y
Thu Jan 29 00:00:00 EST 2015
An acetylation switch controls TDP-43 function and aggregation propensity.
Nature communications
Cohen TJ,Hwang AW,Restrepo CR,Yuan CX,Trojanowski JQ,Lee VM
Mon Jan 05 00:00:00 EST 2015
An acetylation switch controls TDP-43 function and aggregation propensity.
Nature communications
Cohen TJ,Hwang AW,Restrepo CR,Yuan CX,Trojanowski JQ,Lee VM
Mon Jan 05 00:00:00 EST 2015
Hippocampal sclerosis in Lewy body disease is a TDP-43 proteinopathy similar to FTLD-TDP Type A.
Acta neuropathologica
Aoki N,Murray ME,Ogaki K,Fujioka S,Rutherford NJ,Rademakers R,Ross OA,Dickson DW
Thu Jan 01 00:00:00 EST 2015
TDP-43 loss of cellular function through aggregation requires additional structural determinants beyond its C-terminal Q/N prion-like domain.
Human molecular genetics
Budini M,Romano V,Quadri Z,Buratti E,Baralle FE
Thu Jan 01 00:00:00 EST 2015
TDP-43 loss of cellular function through aggregation requires additional structural determinants beyond its C-terminal Q/N prion-like domain.
Human molecular genetics
Budini M,Romano V,Quadri Z,Buratti E,Baralle FE
Thu Jan 01 00:00:00 EST 2015
TDP-43 pathology in the basal forebrain and hypothalamus of patients with amyotrophic lateral sclerosis.
Acta neuropathologica communications
Cykowski MD,Takei H,Schulz PE,Appel SH,Powell SZ
Wed Dec 24 00:00:00 EST 2014
Allele-specific knockdown of ALS-associated mutant TDP-43 in neural stem cells derived from induced pluripotent stem cells.
PloS one
Nishimura AL,Shum C,Scotter EL,Abdelgany A,Sardone V,Wright J,Lee YB,Chen HJ,Bilican B,Carrasco M,Maniatis T,Chandran S,Rogelj B,Gallo JM,Shaw CE
Mon Dec 22 00:00:00 EST 2014
Allele-specific knockdown of ALS-associated mutant TDP-43 in neural stem cells derived from induced pluripotent stem cells.
PloS one
Nishimura AL,Shum C,Scotter EL,Abdelgany A,Sardone V,Wright J,Lee YB,Chen HJ,Bilican B,Carrasco M,Maniatis T,Chandran S,Rogelj B,Gallo JM,Shaw CE
Mon Dec 22 00:00:00 EST 2014
An ALS-mutant TDP-43 neurotoxic peptide adopts an anti-parallel β-structure and induces TDP-43 redistribution.
Human molecular genetics
Zhu L,Xu M,Yang M,Yang Y,Li Y,Deng J,Ruan L,Liu J,Du S,Liu X,Feng W,Fushimi K,Bigio EH,Mesulam M,Wang C,Wu JY
Sat Dec 20 00:00:00 EST 2014
An ALS-mutant TDP-43 neurotoxic peptide adopts an anti-parallel β-structure and induces TDP-43 redistribution.
Human molecular genetics
Zhu L,Xu M,Yang M,Yang Y,Li Y,Deng J,Ruan L,Liu J,Du S,Liu X,Feng W,Fushimi K,Bigio EH,Mesulam M,Wang C,Wu JY
Sat Dec 20 00:00:00 EST 2014
Aggregation properties of the small nuclear ribonucleoprotein U1-70K in Alzheimer disease.
The Journal of biological chemistry
Diner I,Hales CM,Bishof I,Rabenold L,Duong DM,Yi H,Laur O,Gearing M,Troncoso J,Thambisetty M,Lah JJ,Levey AI,Seyfried NT
Fri Dec 19 00:00:00 EST 2014
ALS-associated TDP-43 induces endoplasmic reticulum stress, which drives cytoplasmic TDP-43 accumulation and stress granule formation.
PloS one
Walker AK,Soo KY,Sundaramoorthy V,Parakh S,Ma Y,Farg MA,Wallace RH,Crouch PJ,Turner BJ,Horne MK,Atkin JD
Wed Dec 17 00:00:00 EST 2014
Proteasome dysfunction induces muscle growth defects and protein aggregation.
Journal of cell science
Kitajima Y,Tashiro Y,Suzuki N,Warita H,Kato M,Tateyama M,Ando R,Izumi R,Yamazaki M,Abe M,Sakimura K,Ito H,Urushitani M,Nagatomi R,Takahashi R,Aoki M
Mon Dec 15 00:00:00 EST 2014
Proteasome dysfunction induces muscle growth defects and protein aggregation.
Journal of cell science
Kitajima Y,Tashiro Y,Suzuki N,Warita H,Kato M,Tateyama M,Ando R,Izumi R,Yamazaki M,Abe M,Sakimura K,Ito H,Urushitani M,Nagatomi R,Takahashi R,Aoki M
Mon Dec 15 00:00:00 EST 2014
Immunoreactivity of valosin-containing protein in sporadic amyotrophic lateral sclerosis and in a case of its novel mutant.
Acta neuropathologica communications
Ayaki T,Ito H,Fukushima H,Inoue T,Kondo T,Ikemoto A,Asano T,Shodai A,Fujita T,Fukui S,Morino H,Nakano S,Kusaka H,Yamashita H,Ihara M,Matsumoto R,Kawamata J,Urushitani M,Kawakami H,Takahashi R
Wed Dec 10 00:00:00 EST 2014
H63D HFE genotype accelerates disease progression in animal models of amyotrophic lateral sclerosis.
Biochimica et biophysica acta
Nandar W,Neely EB,Simmons Z,Connor JR
Mon Dec 01 00:00:00 EST 2014
No interaction between tau and TDP-43 pathologies in either frontotemporal lobar degeneration or motor neurone disease.
Neuropathology and applied neurobiology
Robinson AC,Thompson JC,Weedon L,Rollinson S,Pickering-Brown S,Snowden JS,Davidson YS,Mann DM
Mon Dec 01 00:00:00 EST 2014
Cytoplasmic TDP-43 accumulation in cells of the adrenal medulla in individuals with or without amyotrophic lateral sclerosis.
Neuropathology : official journal of the Japanese Society of Neuropathology
Okamoto K,Amari M,Fujita Y,Makioka K,Fukuda T,Suzuki K,Takatama M
Mon Dec 01 00:00:00 EST 2014
Neuropathological features of genetically confirmed DYT1 dystonia: investigating disease-specific inclusions.
Acta neuropathologica communications
Paudel R,Kiely A,Li A,Lashley T,Bandopadhyay R,Hardy J,Jinnah HA,Bhatia K,Houlden H,Holton JL
Tue Nov 18 00:00:00 EST 2014
CGG repeats in RNA modulate expression of TDP-43 in mouse and fly models of fragile X tremor ataxia syndrome.
Human molecular genetics
Galloway JN,Shaw C,Yu P,Parghi D,Poidevin M,Jin P,Nelson DL
Sat Nov 15 00:00:00 EST 2014
Divergent phenotypes in mutant TDP-43 transgenic mice highlight potential confounds in TDP-43 transgenic modeling.
PloS one
D'Alton S,Altshuler M,Cannon A,Dickson DW,Petrucelli L,Lewis J
Tue Nov 04 00:00:00 EST 2014
Concomitant accumulation of α-synuclein and TDP-43 in a patient with corticobasal degeneration.
Journal of neurology
Yamashita S,Sakashita N,Yamashita T,Tawara N,Tasaki M,Kawakami K,Komohara Y,Fujiwara Y,Kamikawa M,Nakagawa T,Hirano T,Maeda Y,Hasegawa M,Takeya M,Ando Y
Sat Nov 01 00:00:00 EDT 2014
Intrathecal infusion of BMAA induces selective motor neuron damage and astrogliosis in the ventral horn of the spinal cord.
Experimental neurology
Yin HZ,Yu S,Hsu CI,Liu J,Acab A,Wu R,Tao A,Chiang BJ,Weiss JH
Sat Nov 01 00:00:00 EDT 2014
ALS-associated peripherin spliced transcripts form distinct protein inclusions that are neuroprotective against oxidative stress.
Experimental neurology
McLean JR,Smith GA,Rocha EM,Osborn TM,Dib S,Hayes MA,Beagan JA,Brown TB,Lawson TF,Hallett PJ,Robertson J,Isacson O
Sat Nov 01 00:00:00 EDT 2014
ALS-associated peripherin spliced transcripts form distinct protein inclusions that are neuroprotective against oxidative stress.
Experimental neurology
McLean JR,Smith GA,Rocha EM,Osborn TM,Dib S,Hayes MA,Beagan JA,Brown TB,Lawson TF,Hallett PJ,Robertson J,Isacson O
Sat Nov 01 00:00:00 EDT 2014
Loss of nuclear TDP-43 in amyotrophic lateral sclerosis (ALS) causes altered expression of splicing machinery and widespread dysregulation of RNA splicing in motor neurones.
Neuropathology and applied neurobiology
Highley JR,Kirby J,Jansweijer JA,Webb PS,Hewamadduma CA,Heath PR,Higginbottom A,Raman R,Ferraiuolo L,Cooper-Knock J,McDermott CJ,Wharton SB,Shaw PJ,Ince PG
Wed Oct 01 00:00:00 EDT 2014
Loss of nuclear TDP-43 in amyotrophic lateral sclerosis (ALS) causes altered expression of splicing machinery and widespread dysregulation of RNA splicing in motor neurones.
Neuropathology and applied neurobiology
Highley JR,Kirby J,Jansweijer JA,Webb PS,Hewamadduma CA,Heath PR,Higginbottom A,Raman R,Ferraiuolo L,Cooper-Knock J,McDermott CJ,Wharton SB,Shaw PJ,Ince PG
Wed Oct 01 00:00:00 EDT 2014
Potentiated Hsp104 variants suppress toxicity of diverse neurodegenerative disease-linked proteins.
Disease models & mechanisms
Jackrel ME,Shorter J
Wed Oct 01 00:00:00 EDT 2014
TDP-43 suppresses CGG repeat-induced neurotoxicity through interactions with HnRNP A2/B1.
Human molecular genetics
He F,Krans A,Freibaum BD,Taylor JP,Todd PK
Wed Oct 01 00:00:00 EDT 2014
TDP-43 suppresses CGG repeat-induced neurotoxicity through interactions with HnRNP A2/B1.
Human molecular genetics
He F,Krans A,Freibaum BD,Taylor JP,Todd PK
Wed Oct 01 00:00:00 EDT 2014
RNA-processing protein TDP-43 regulates FOXO-dependent protein quality control in stress response.
PLoS genetics
Zhang T,Baldie G,Periz G,Wang J
Wed Oct 01 00:00:00 EDT 2014
RNA-processing protein TDP-43 regulates FOXO-dependent protein quality control in stress response.
PLoS genetics
Zhang T,Baldie G,Periz G,Wang J
Wed Oct 01 00:00:00 EDT 2014
Hippocampal sclerosis dementia with the C9ORF72 hexanucleotide repeat expansion.
Neurobiology of aging
Pletnikova O,Sloane KL,Renton AE,Traynor BJ,Crain BJ,Reid T,Zu T,Ranum LP,Troncoso JC,Rabins PV,Onyike CU
Wed Oct 01 00:00:00 EDT 2014
The co-occurrence of Alzheimer's disease and Huntington's disease: a neuropathological study of 15 elderly Huntington's disease subjects.
Journal of Huntington's disease
Davis MY,Keene CD,Jayadev S,Bird T
Tue Sep 23 00:00:00 EDT 2014
Early retinal neurodegeneration and impaired Ran-mediated nuclear import of TDP-43 in progranulin-deficient FTLD.
The Journal of experimental medicine
Ward ME,Taubes A,Chen R,Miller BL,Sephton CF,Gelfand JM,Minami S,Boscardin J,Martens LH,Seeley WW,Yu G,Herz J,Filiano AJ,Arrant AE,Roberson ED,Kraft TW,Farese RV,Green A,Gan L
Mon Sep 22 00:00:00 EDT 2014
Early retinal neurodegeneration and impaired Ran-mediated nuclear import of TDP-43 in progranulin-deficient FTLD.
The Journal of experimental medicine
Ward ME,Taubes A,Chen R,Miller BL,Sephton CF,Gelfand JM,Minami S,Boscardin J,Martens LH,Seeley WW,Yu G,Herz J,Filiano AJ,Arrant AE,Roberson ED,Kraft TW,Farese RV,Green A,Gan L
Mon Sep 22 00:00:00 EDT 2014
Parkin-mediated reduction of nuclear and soluble TDP-43 reverses behavioral decline in symptomatic mice.
Human molecular genetics
Wenqiang C,Lonskaya I,Hebron ML,Ibrahim Z,Olszewski RT,Neale JH,Moussa CE
Mon Sep 15 00:00:00 EDT 2014
Parkin-mediated reduction of nuclear and soluble TDP-43 reverses behavioral decline in symptomatic mice.
Human molecular genetics
Wenqiang C,Lonskaya I,Hebron ML,Ibrahim Z,Olszewski RT,Neale JH,Moussa CE
Mon Sep 15 00:00:00 EDT 2014
Brain injection of α-synuclein induces multiple proteinopathies, gliosis, and a neuronal injury marker.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Sacino AN,Brooks M,McKinney AB,Thomas MA,Shaw G,Golde TE,Giasson BI
Wed Sep 10 00:00:00 EDT 2014
TDP-43 toxicity proceeds via calcium dysregulation and necrosis in aging Caenorhabditis elegans motor neurons.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Aggad D,Vérièpe J,Tauffenberger A,Parker JA
Wed Sep 03 00:00:00 EDT 2014
Corticobasal degeneration initially developing motor versus non-motor symptoms: a comparative clinicopathological study.
Psychogeriatrics : the official journal of the Japanese Psychogeriatric Society
Ikeda C,Yokota O,Nagao S,Ishizu H,Morisada Y,Terada S,Nakashima Y,Akiyama H,Uchitomi Y
Mon Sep 01 00:00:00 EDT 2014
Atypical Alzheimer's disease in an elderly United States resident with amyotrophic lateral sclerosis and pathological tau in spinal motor neurons.
Amyotrophic lateral sclerosis & frontotemporal degeneration
McCluskey LF,Geser F,Elman LB,Van Deerlin VM,Robinson JL,Lee VM,Trojanowski JQ
Mon Sep 01 00:00:00 EDT 2014
Dual vulnerability of TDP-43 to calpain and caspase-3 proteolysis after neurotoxic conditions and traumatic brain injury.
Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism
Yang Z,Lin F,Robertson CS,Wang KK
Mon Sep 01 00:00:00 EDT 2014
Dual vulnerability of TDP-43 to calpain and caspase-3 proteolysis after neurotoxic conditions and traumatic brain injury.
Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism
Yang Z,Lin F,Robertson CS,Wang KK
Mon Sep 01 00:00:00 EDT 2014
RNA metabolism in ALS: when normal processes become pathological.
Amyotrophic lateral sclerosis & frontotemporal degeneration
Droppelmann CA,Campos-Melo D,Ishtiaq M,Volkening K,Strong MJ
Mon Sep 01 00:00:00 EDT 2014
Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models.
Nature chemical biology
Barmada SJ,Serio A,Arjun A,Bilican B,Daub A,Ando DM,Tsvetkov A,Pleiss M,Li X,Peisach D,Shaw C,Chandran S,Finkbeiner S
Fri Aug 01 00:00:00 EDT 2014
New criteria for frontotemporal dementia syndromes: clinical and pathological diagnostic implications.
Journal of neurology, neurosurgery, and psychiatry
Chare L,Hodges JR,Leyton CE,McGinley C,Tan RH,Kril JJ,Halliday GM
Fri Aug 01 00:00:00 EDT 2014
The voltage-gated calcium channel blocker lomerizine is neuroprotective in motor neurons expressing mutant SOD1, but not TDP-43.
Journal of neurochemistry
Tran LT,Gentil BJ,Sullivan KE,Durham HD
Fri Aug 01 00:00:00 EDT 2014
The voltage-gated calcium channel blocker lomerizine is neuroprotective in motor neurons expressing mutant SOD1, but not TDP-43.
Journal of neurochemistry
Tran LT,Gentil BJ,Sullivan KE,Durham HD
Fri Aug 01 00:00:00 EDT 2014
Myopathy-causing mutations in an HSP40 chaperone disrupt processing of specific client conformers.
The Journal of biological chemistry
Stein KC,Bengoechea R,Harms MB,Weihl CC,True HL
Fri Jul 25 00:00:00 EDT 2014
Possible involvement of lysosomal dysfunction in pathological changes of the brain in aged progranulin-deficient mice.
Acta neuropathologica communications
Tanaka Y,Chambers JK,Matsuwaki T,Yamanouchi K,Nishihara M
Tue Jul 15 00:00:00 EDT 2014
Metabolism and mis-metabolism of the neuropathological signature protein TDP-43.
Journal of cell science
Huang CC,Bose JK,Majumder P,Lee KH,Huang JT,Huang JK,Shen CK
Tue Jul 15 00:00:00 EDT 2014
Metabolism and mis-metabolism of the neuropathological signature protein TDP-43.
Journal of cell science
Huang CC,Bose JK,Majumder P,Lee KH,Huang JT,Huang JK,Shen CK
Tue Jul 15 00:00:00 EDT 2014
RPL24: a potential therapeutic target whose depletion or acetylation inhibits polysome assembly and cancer cell growth.
Oncotarget
Wilson-Edell KA,Kehasse A,Scott GK,Yau C,Rothschild DE,Schilling B,Gabriel BS,Yevtushenko MA,Hanson IM,Held JM,Gibson BW,Benz CC
Tue Jul 15 00:00:00 EDT 2014
UBE2E ubiquitin-conjugating enzymes and ubiquitin isopeptidase Y regulate TDP-43 protein ubiquitination.
The Journal of biological chemistry
Hans F,Fiesel FC,Strong JC,Jäckel S,Rasse TM,Geisler S,Springer W,Schulz JB,Voigt A,Kahle PJ
Fri Jul 04 00:00:00 EDT 2014
UBE2E ubiquitin-conjugating enzymes and ubiquitin isopeptidase Y regulate TDP-43 protein ubiquitination.
The Journal of biological chemistry
Hans F,Fiesel FC,Strong JC,Jäckel S,Rasse TM,Geisler S,Springer W,Schulz JB,Voigt A,Kahle PJ
Fri Jul 04 00:00:00 EDT 2014
Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansions.
Brain : a journal of neurology
Cooper-Knock J,Walsh MJ,Higginbottom A,Robin Highley J,Dickman MJ,Edbauer D,Ince PG,Wharton SB,Wilson SA,Kirby J,Hautbergue GM,Shaw PJ
Tue Jul 01 00:00:00 EDT 2014
Abnormal distribution of heterogeneous nuclear ribonucleoproteins in sporadic inclusion body myositis.
Neuromuscular disorders : NMD
Pinkus JL,Amato AA,Taylor JP,Greenberg SA
Tue Jul 01 00:00:00 EDT 2014
Neuropathologic assessment of dementia markers in identical and fraternal twins.
Brain pathology (Zurich, Switzerland)
Iacono D,Volkman I,Nennesmo I,Pedersen NL,Fratiglioni L,Johansson B,Karlsson D,Winblad B,Gatz M
Tue Jul 01 00:00:00 EDT 2014
Mitochondrial dysfunction and decrease in body weight of transgenic knock-in mouse model for TDP-43: the question of glucose?
The Journal of biological chemistry
Jawaid A,Gapp K,Schulz PE
Fri Jun 27 00:00:00 EDT 2014
Mitochondrial dysfunction and decrease in body weight of transgenic knock-in mouse model for TDP-43: the question of glucose?
The Journal of biological chemistry
Jawaid A,Gapp K,Schulz PE
Fri Jun 27 00:00:00 EDT 2014
Brain distribution of dipeptide repeat proteins in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72.
Acta neuropathologica communications
Davidson YS,Barker H,Robinson AC,Thompson JC,Harris J,Troakes C,Smith B,Al-Saraj S,Shaw C,Rollinson S,Masuda-Suzukake M,Hasegawa M,Pickering-Brown S,Snowden JS,Mann DM
Fri Jun 20 00:00:00 EDT 2014
Downregulation of microRNA-9 in iPSC-derived neurons of FTD/ALS patients with TDP-43 mutations.
PloS one
Zhang Z,Almeida S,Lu Y,Nishimura AL,Peng L,Sun D,Wu B,Karydas AM,Tartaglia MC,Fong JC,Miller BL,Farese RV,Moore MJ,Shaw CE,Gao FB
Tue Jun 17 00:00:00 EDT 2014
Downregulation of microRNA-9 in iPSC-derived neurons of FTD/ALS patients with TDP-43 mutations.
PloS one
Zhang Z,Almeida S,Lu Y,Nishimura AL,Peng L,Sun D,Wu B,Karydas AM,Tartaglia MC,Fong JC,Miller BL,Farese RV,Moore MJ,Shaw CE,Gao FB
Tue Jun 17 00:00:00 EDT 2014
Partial deletion of AFG3L2 causing spinocerebellar ataxia type 28.
Neurology
Smets K,Deconinck T,Baets J,Sieben A,Martin JJ,Smouts I,Wang S,Taroni F,Di Bella D,Van Hecke W,Parizel PM,Jadoul C,De Potter R,Couvreur F,Rugarli E,De Jonghe P
Tue Jun 10 00:00:00 EDT 2014
FUS is phosphorylated by DNA-PK and accumulates in the cytoplasm after DNA damage.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Deng Q,Holler CJ,Taylor G,Hudson KF,Watkins W,Gearing M,Ito D,Murray ME,Dickson DW,Seyfried NT,Kukar T
Wed Jun 04 00:00:00 EDT 2014
Frontotemporal lobar degeneration with writing disturbance mainly consisting of omission of kana letters.
Neurocase
Shimizu T,Mochizuki Y,Bandoh M,Taguchi T,Mishima K,Takeda K,Nojima K,Mizutani T,Matsubara S
Sun Jun 01 00:00:00 EDT 2014
Neuropathological features of multiple system atrophy with cognitive impairment.
Movement disorders : official journal of the Movement Disorder Society
Asi YT,Ling H,Ahmed Z,Lees AJ,Revesz T,Holton JL
Sun Jun 01 00:00:00 EDT 2014
Plasma phosphorylated TDP-43 levels are elevated in patients with frontotemporal dementia carrying a C9orf72 repeat expansion or a GRN mutation.
Journal of neurology, neurosurgery, and psychiatry
Suárez-Calvet M,Dols-Icardo O,Lladó A,Sánchez-Valle R,Hernández I,Amer G,Antón-Aguirre S,Alcolea D,Fortea J,Ferrer I,van der Zee J,Dillen L,Van Broeckhoven C,Molinuevo JL,Blesa R,Clarimón J,Lleó A
Sun Jun 01 00:00:00 EDT 2014
Argyrophilic grain disease as a neurodegenerative substrate in late-onset schizophrenia and delusional disorders.
European archives of psychiatry and clinical neuroscience
Nagao S,Yokota O,Ikeda C,Takeda N,Ishizu H,Kuroda S,Sudo K,Terada S,Murayama S,Uchitomi Y
Sun Jun 01 00:00:00 EDT 2014
TAR DNA-binding protein 43 pathology in Alzheimer's disease with psychosis.
International psychogeriatrics
Vatsavayi AV,Kofler J,Demichele-Sweet MA,Murray PS,Lopez OL,Sweet RA
Sun Jun 01 00:00:00 EDT 2014
The RNA-binding protein TDP-43 selectively disrupts microRNA-1/206 incorporation into the RNA-induced silencing complex.
The Journal of biological chemistry
King IN,Yartseva V,Salas D,Kumar A,Heidersbach A,Ando DM,Stallings NR,Elliott JL,Srivastava D,Ivey KN
Fri May 16 00:00:00 EDT 2014
The RNA-binding protein TDP-43 selectively disrupts microRNA-1/206 incorporation into the RNA-induced silencing complex.
The Journal of biological chemistry
King IN,Yartseva V,Salas D,Kumar A,Heidersbach A,Ando DM,Stallings NR,Elliott JL,Srivastava D,Ivey KN
Fri May 16 00:00:00 EDT 2014
Differential motor neuron involvement in progressive muscular atrophy: a comparative study with amyotrophic lateral sclerosis.
BMJ open
Riku Y,Atsuta N,Yoshida M,Tatsumi S,Iwasaki Y,Mimuro M,Watanabe H,Ito M,Senda J,Nakamura R,Koike H,Sobue G
Wed May 14 00:00:00 EDT 2014
TDP-43, an ALS linked protein, regulates fat deposition and glucose homeostasis.
PloS one
Stallings NR,Puttaparthi K,Dowling KJ,Luther CM,Burns DK,Davis K,Elliott JL
Wed May 14 00:00:00 EDT 2014
Astrocytic TDP-43 pathology in Alexander disease.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Walker AK,Daniels CM,Goldman JE,Trojanowski JQ,Lee VM,Messing A
Wed May 07 00:00:00 EDT 2014
Prevalence of dementia subtypes in a developing country: a clinicopathological study.
Clinics (Sao Paulo, Brazil)
Grinberg LT,Nitrini R,Suemoto CK,Lucena Ferretti-Rebustini RE,Leite RE,Farfel JM,Santos E,Andrade MP,Alho AT,Lima Mdo C,Oliveira KC,Tampellini E,Polichiso L,Santos GB,Rodriguez RD,Ueda K,Pasqualucci CA,Jacob-Filho W
Fri May 02 00:00:00 EDT 2014
Tar DNA-binding protein-43 (TDP-43) regulates axon growth in vitro and in vivo.
Neurobiology of disease
Tripathi VB,Baskaran P,Shaw CE,Guthrie S
Thu May 01 00:00:00 EDT 2014
Tar DNA-binding protein-43 (TDP-43) regulates axon growth in vitro and in vivo.
Neurobiology of disease
Tripathi VB,Baskaran P,Shaw CE,Guthrie S
Thu May 01 00:00:00 EDT 2014
Differential diagnosis of amyotrophic lateral sclerosis from Guillain-Barré syndrome by quantitative determination of TDP-43 in cerebrospinal fluid.
The International journal of neuroscience
Hosokawa M,Arai T,Yamashita M,Tsuji H,Nonaka T,Masuda-Suzukake M,Tamaoka A,Hasegawa M,Akiyama H
Thu May 01 00:00:00 EDT 2014
Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis.
Nature neuroscience
Johnson JO,Pioro EP,Boehringer A,Chia R,Feit H,Renton AE,Pliner HA,Abramzon Y,Marangi G,Winborn BJ,Gibbs JR,Nalls MA,Morgan S,Shoai M,Hardy J,Pittman A,Orrell RW,Malaspina A,Sidle KC,Fratta P,Harms MB,Baloh RH,Pestronk A,Weihl CC,Rogaeva E,Zinman L,Drory VE,Borghero G,Mora G,Calvo A,Rothstein JD,Drepper C,Sendtner M,Singleton AB,Taylor JP,Cookson MR,Restagno G,Sabatelli M,Bowser R,Chiò A,Traynor BJ
Thu May 01 00:00:00 EDT 2014
Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis.
Nature neuroscience
Johnson JO,Pioro EP,Boehringer A,Chia R,Feit H,Renton AE,Pliner HA,Abramzon Y,Marangi G,Winborn BJ,Gibbs JR,Nalls MA,Morgan S,Shoai M,Hardy J,Pittman A,Orrell RW,Malaspina A,Sidle KC,Fratta P,Harms MB,Baloh RH,Pestronk A,Weihl CC,Rogaeva E,Zinman L,Drory VE,Borghero G,Mora G,Calvo A,Rothstein JD,Drepper C,Sendtner M,Singleton AB,Taylor JP,Cookson MR,Restagno G,Sabatelli M,Bowser R,Chiò A,Traynor BJ
Thu May 01 00:00:00 EDT 2014
PRKAR1B mutation associated with a new neurodegenerative disorder with unique pathology.
Brain : a journal of neurology
Wong TH,Chiu WZ,Breedveld GJ,Li KW,Verkerk AJ,Hondius D,Hukema RK,Seelaar H,Frick P,Severijnen LA,Lammers GJ,Lebbink JH,van Duinen SG,Kamphorst W,Rozemuller AJ,Bakker EB,Neumann M,Willemsen R,Bonifati V,Smit AB,van Swieten J
Thu May 01 00:00:00 EDT 2014
A novel GRN mutation (GRN c.708+6_+9delTGAG) in frontotemporal lobar degeneration with TDP-43-positive inclusions: clinicopathologic report of 6 cases.
Journal of neuropathology and experimental neurology
Bit-Ivan EN,Suh E,Shim HS,Weintraub S,Hyman BT,Arnold SE,McCarty-Wood E,Van Deerlin VM,Schneider JA,Trojanowski JQ,Frosch MP,Baker MC,Rademakers R,Mesulam M,Bigio EH
Thu May 01 00:00:00 EDT 2014
A retrospective cohort study identifying the principal pathological features useful in the diagnosis of inclusion body myositis.
BMJ open
Brady S,Squier W,Sewry C,Hanna M,Hilton-Jones D,Holton JL
Mon Apr 28 00:00:00 EDT 2014
Neuropathology of partial PGC-1α deficiency recapitulates features of mitochondrial encephalopathies but not of neurodegenerative diseases.
Neuro-degenerative diseases
Szalardy L,Zadori D,Plangar I,Vecsei L,Weydt P,Ludolph AC,Klivenyi P,Kovacs GG
Mon Apr 21 00:00:00 EDT 2014
A novel approach for integrative studies on neurodegenerative diseases in human brains.
Journal of neuroscience methods
Theofilas P,Polichiso L,Wang X,Lima LC,Alho ATL,Leite REP,Suemoto CK,Pasqualucci CA,Jacob-Filho W,Heinsen H,Grinberg LT
Tue Apr 15 00:00:00 EDT 2014
Mitochondrial dysfunction and decrease in body weight of a transgenic knock-in mouse model for TDP-43.
The Journal of biological chemistry
Stribl C,Samara A,Trümbach D,Peis R,Neumann M,Fuchs H,Gailus-Durner V,Hrabě de Angelis M,Rathkolb B,Wolf E,Beckers J,Horsch M,Neff F,Kremmer E,Koob S,Reichert AS,Hans W,Rozman J,Klingenspor M,Aichler M,Walch AK,Becker L,Klopstock T,Glasl L,Hölter SM,Wurst W,Floss T
Fri Apr 11 00:00:00 EDT 2014
Mitochondrial dysfunction and decrease in body weight of a transgenic knock-in mouse model for TDP-43.
The Journal of biological chemistry
Stribl C,Samara A,Trümbach D,Peis R,Neumann M,Fuchs H,Gailus-Durner V,Hrabě de Angelis M,Rathkolb B,Wolf E,Beckers J,Horsch M,Neff F,Kremmer E,Koob S,Reichert AS,Hans W,Rozman J,Klingenspor M,Aichler M,Walch AK,Becker L,Klopstock T,Glasl L,Hölter SM,Wurst W,Floss T
Fri Apr 11 00:00:00 EDT 2014
Parkin reverses TDP-43-induced cell death and failure of amino acid homeostasis.
Journal of neurochemistry
Hebron M,Chen W,Miessau MJ,Lonskaya I,Moussa CE
Tue Apr 01 00:00:00 EDT 2014
Accumulation of the sigma-1 receptor is common to neuronal nuclear inclusions in various neurodegenerative diseases.
Neuropathology : official journal of the Japanese Society of Neuropathology
Miki Y,Mori F,Kon T,Tanji K,Toyoshima Y,Yoshida M,Sasaki H,Kakita A,Takahashi H,Wakabayashi K
Tue Apr 01 00:00:00 EDT 2014
Diagnosis and management of behavioral variant frontotemporal dementia.
Biological psychiatry
Pressman PS,Miller BL
Tue Apr 01 00:00:00 EDT 2014
FUS colocalizes with polyglutamine, but not with TDP-43 in neuronal intranuclear inclusions in spinocerebellar ataxia type 2.
Neuropathology and applied neurobiology
Mori F,Toyoshima Y,Tanji K,Kakita A,Takahashi H,Wakabayashi K
Tue Apr 01 00:00:00 EDT 2014
Ubiquitin-negative, eosinophilic neuronal cytoplasmic inclusions associated with stress granules and autophagy: an immunohistochemical investigation of two cases.
Neuropathology : official journal of the Japanese Society of Neuropathology
Mori F,Watanabe Y,Miki Y,Tanji K,Odagiri S,Eto K,Wakabayashi K
Tue Apr 01 00:00:00 EDT 2014
Reduced cellular Ca(2+) availability enhances TDP-43 cleavage by apoptotic caspases.
Biochimica et biophysica acta
De Marco G,Lomartire A,Mandili G,Lupino E,Buccinnà B,Ramondetti C,Moglia C,Novelli F,Piccinini M,Mostert M,Rinaudo MT,Chiò A,Calvo A
Tue Apr 01 00:00:00 EDT 2014
ALS-linked mutations enlarge TDP-43-enriched neuronal RNA granules in the dendritic arbor.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Liu-Yesucevitz L,Lin AY,Ebata A,Boon JY,Reid W,Xu YF,Kobrin K,Murphy GJ,Petrucelli L,Wolozin B
Wed Mar 19 00:00:00 EDT 2014
Differential roles of the ubiquitin proteasome system and autophagy in the clearance of soluble and aggregated TDP-43 species.
Journal of cell science
Scotter EL,Vance C,Nishimura AL,Lee YB,Chen HJ,Urwin H,Sardone V,Mitchell JC,Rogelj B,Rubinsztein DC,Shaw CE
Sat Mar 15 00:00:00 EDT 2014
Staging TDP-43 pathology in Alzheimer's disease.
Acta neuropathologica
Josephs KA,Murray ME,Whitwell JL,Parisi JE,Petrucelli L,Jack CR,Petersen RC,Dickson DW
Sat Mar 01 00:00:00 EST 2014
Early dipeptide repeat pathology in a frontotemporal dementia kindred with C9ORF72 mutation and intellectual disability.
Acta neuropathologica
Proudfoot M,Gutowski NJ,Edbauer D,Hilton DA,Stephens M,Rankin J,Mackenzie IR
Sat Mar 01 00:00:00 EST 2014
Hereditary myopathy with early respiratory failure: occurrence in various populations.
Journal of neurology, neurosurgery, and psychiatry
Palmio J,Evilä A,Chapon F,Tasca G,Xiang F,Brådvik B,Eymard B,Echaniz-Laguna A,Laporte J,Kärppä M,Mahjneh I,Quinlivan R,Laforêt P,Damian M,Berardo A,Taratuto AL,Bueri JA,Tommiska J,Raivio T,Tuerk M,Gölitz P,Chevessier F,Sewry C,Norwood F,Hedberg C,Schröder R,Edström L,Oldfors A,Hackman P,Udd B
Sat Mar 01 00:00:00 EST 2014
Autopsy case of concurrent Huntington's disease and neurofibromatosis type 1.
Psychogeriatrics : the official journal of the Japanese Psychogeriatric Society
Kawakami I,Katsuse O,Aoki N,Togo T,Suzuki K,Isojima D,Kondo D,Iseki E,Kosaka K,Akiyama H,Hirayasu Y
Sat Mar 01 00:00:00 EST 2014
Increased metal content in the TDP-43(A315T) transgenic mouse model of frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Frontiers in aging neuroscience
Dang TN,Lim NK,Grubman A,Li QX,Volitakis I,White AR,Crouch PJ
Thu Feb 27 00:00:00 EST 2014
NF-Y inactivation causes atypical neurodegeneration characterized by ubiquitin and p62 accumulation and endoplasmic reticulum disorganization.
Nature communications
Yamanaka T,Tosaki A,Kurosawa M,Matsumoto G,Koike M,Uchiyama Y,Maity SN,Shimogori T,Hattori N,Nukina N
Tue Feb 25 00:00:00 EST 2014
TDP-43 Phosphorylation by casein kinase Iε promotes oligomerization and enhances toxicity in vivo.
Human molecular genetics
Choksi DK,Roy B,Chatterjee S,Yusuff T,Bakhoum MF,Sengupta U,Ambegaokar S,Kayed R,Jackson GR
Sat Feb 15 00:00:00 EST 2014
TDP-43 Phosphorylation by casein kinase Iε promotes oligomerization and enhances toxicity in vivo.
Human molecular genetics
Choksi DK,Roy B,Chatterjee S,Yusuff T,Bakhoum MF,Sengupta U,Ambegaokar S,Kayed R,Jackson GR
Sat Feb 15 00:00:00 EST 2014
Lower motor neuron involvement in TAR DNA-binding protein of 43 kDa-related frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
JAMA neurology
Riku Y,Watanabe H,Yoshida M,Tatsumi S,Mimuro M,Iwasaki Y,Katsuno M,Iguchi Y,Masuda M,Senda J,Ishigaki S,Udagawa T,Sobue G
Sat Feb 01 00:00:00 EST 2014
De novo prion aggregates trigger autophagy in skeletal muscle.
Journal of virology
Joshi-Barr S,Bett C,Chiang WC,Trejo M,Goebel HH,Sikorska B,Liberski P,Raeber A,Lin JH,Masliah E,Sigurdson CJ
Sat Feb 01 00:00:00 EST 2014
De novo prion aggregates trigger autophagy in skeletal muscle.
Journal of virology
Joshi-Barr S,Bett C,Chiang WC,Trejo M,Goebel HH,Sikorska B,Liberski P,Raeber A,Lin JH,Masliah E,Sigurdson CJ
Sat Feb 01 00:00:00 EST 2014
Therapeutic modulation of eIF2α phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models.
Nature genetics
Kim HJ,Raphael AR,LaDow ES,McGurk L,Weber RA,Trojanowski JQ,Lee VM,Finkbeiner S,Gitler AD,Bonini NM
Sat Feb 01 00:00:00 EST 2014
Clinicopathologic features of autosomal recessive amyotrophic lateral sclerosis associated with optineurin mutation.
Neuropathology : official journal of the Japanese Society of Neuropathology
Kamada M,Izumi Y,Ayaki T,Nakamura M,Kagawa S,Kudo E,Sako W,Maruyama H,Nishida Y,Kawakami H,Ito H,Kaji R
Sat Feb 01 00:00:00 EST 2014
Dendritic retraction, but not atrophy, is consistent in amyotrophic lateral sclerosis-comparison between Onuf's neurons and other sacral motor neurons.
Acta neuropathologica communications
Takeda T,Uchihara T,Nakayama Y,Nakamura A,Sasaki S,Kakei S,Uchiyama S,Duyckaerts C,Yoshida M
Mon Jan 27 00:00:00 EST 2014
ApoE and TDP-43 neuropathology in two siblings with familial FTLD-motor neuron disease.
Neurocase
Vossel KA,Bien-Ly N,Bernardo A,Rascovsky K,Karydas A,Rabinovici GD,Sidhu M,Huang EJ,Miller BL,Huang Y,Seeley WW
Thu Jan 23 00:00:00 EST 2014
RNP2 of RNA recognition motif 1 plays a central role in the aberrant modification of TDP-43.
PloS one
Takagi S,Iguchi Y,Katsuno M,Ishigaki S,Ikenaka K,Fujioka Y,Honda D,Niwa J,Tanaka F,Watanabe H,Adachi H,Sobue G
Tue Jan 21 00:00:00 EST 2014
RNP2 of RNA recognition motif 1 plays a central role in the aberrant modification of TDP-43.
PloS one
Takagi S,Iguchi Y,Katsuno M,Ishigaki S,Ikenaka K,Fujioka Y,Honda D,Niwa J,Tanaka F,Watanabe H,Adachi H,Sobue G
Tue Jan 21 00:00:00 EST 2014
Potentiated Hsp104 variants antagonize diverse proteotoxic misfolding events.
Cell
Jackrel ME,DeSantis ME,Martinez BA,Castellano LM,Stewart RM,Caldwell KA,Caldwell GA,Shorter J
Thu Jan 16 00:00:00 EST 2014
Titinopathy in a Canadian family sharing the British founder haplotype.
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
Pfeffer G,Joseph JT,Innes AM,Frizzell JB,Wilson IJ,Brownell AK,Chinnery PF
Wed Jan 01 00:00:00 EST 2014
Accumulation of C-terminal fragments of transactive response DNA-binding protein 43 leads to synaptic loss and cognitive deficits in human TDP-43 transgenic mice.
Neurobiology of aging
Medina DX,Orr ME,Oddo S
Wed Jan 01 00:00:00 EST 2014
Accumulation of C-terminal fragments of transactive response DNA-binding protein 43 leads to synaptic loss and cognitive deficits in human TDP-43 transgenic mice.
Neurobiology of aging
Medina DX,Orr ME,Oddo S
Wed Jan 01 00:00:00 EST 2014
Prion-like nuclear aggregation of TDP-43 during heat shock is regulated by HSP40/70 chaperones.
Human molecular genetics
Udan-Johns M,Bengoechea R,Bell S,Shao J,Diamond MI,True HL,Weihl CC,Baloh RH
Wed Jan 01 00:00:00 EST 2014
Prion-like nuclear aggregation of TDP-43 during heat shock is regulated by HSP40/70 chaperones.
Human molecular genetics
Udan-Johns M,Bengoechea R,Bell S,Shao J,Diamond MI,True HL,Weihl CC,Baloh RH
Wed Jan 01 00:00:00 EST 2014
A high-content screen identifies novel compounds that inhibit stress-induced TDP-43 cellular aggregation and associated cytotoxicity.
Journal of biomolecular screening
Boyd JD,Lee P,Feiler MS,Zauur N,Liu M,Concannon J,Ebata A,Wolozin B,Glicksman MA
Wed Jan 01 00:00:00 EST 2014
A 43-kDa TDP-43 species is present in aggregates associated with frontotemporal lobar degeneration.
PloS one
Bosque PJ,Boyer PJ,Mishra P
Mon Dec 30 00:00:00 EST 2013
Pathological features of FTLD-FUS in a Japanese population: analyses of nine cases.
Journal of the neurological sciences
Kobayashi Z,Kawakami I,Arai T,Yokota O,Tsuchiya K,Kondo H,Shimomura Y,Haga C,Aoki N,Hasegawa M,Hosokawa M,Oshima K,Niizato K,Ishizu H,Terada S,Onaya M,Ikeda M,Oyanagi K,Nakano I,Murayama S,Akiyama H,Mizusawa H
Sun Dec 15 00:00:00 EST 2013
Hexanucleotide repeats in ALS/FTD form length-dependent RNA foci, sequester RNA binding proteins, and are neurotoxic.
Cell reports
Lee YB,Chen HJ,Peres JN,Gomez-Deza J,Attig J,Stalekar M,Troakes C,Nishimura AL,Scotter EL,Vance C,Adachi Y,Sardone V,Miller JW,Smith BN,Gallo JM,Ule J,Hirth F,Rogelj B,Houart C,Shaw CE
Thu Dec 12 00:00:00 EST 2013
Clinicopathologic study of Alzheimer's disease: Alzheimer mimics.
Journal of Alzheimer's disease : JAD
Shim YS,Roe CM,Buckles VD,Morris JC
Tue Dec 10 00:00:00 EST 2013
The ALS/FTLD-related RNA-binding proteins TDP-43 and FUS have common downstream RNA targets in cortical neurons.
FEBS open bio
Honda D,Ishigaki S,Iguchi Y,Fujioka Y,Udagawa T,Masuda A,Ohno K,Katsuno M,Sobue G
Mon Dec 09 00:00:00 EST 2013
Neurocognitive speed associates with frontotemporal lobar degeneration TDP-43 subtypes.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
Dean PM,Smith GE,Parisi JE,Dickson DW,Petersen RC,Josephs KA
Sun Dec 01 00:00:00 EST 2013
Increased number of astrocytes and macrophages/microglial cells in the corpus callosum in amyotrophic lateral sclerosis.
Neuropathology : official journal of the Japanese Society of Neuropathology
Sugiyama M,Takao M,Hatsuta H,Funabe S,Ito S,Obi T,Tanaka F,Kuroiwa Y,Murayama S
Sun Dec 01 00:00:00 EST 2013
Cell number changes in Alzheimer's disease relate to dementia, not to plaques and tangles.
Brain : a journal of neurology
Andrade-Moraes CH,Oliveira-Pinto AV,Castro-Fonseca E,da Silva CG,Guimarães DM,Szczupak D,Parente-Bruno DR,Carvalho LR,Polichiso L,Gomes BV,Oliveira LM,Rodriguez RD,Leite RE,Ferretti-Rebustini RE,Jacob-Filho W,Pasqualucci CA,Grinberg LT,Lent R
Sun Dec 01 00:00:00 EST 2013
A clinicopathological and genetic study of sporadic diffuse leukoencephalopathy with spheroids: a report of two cases.
Neuropathology and applied neurobiology
Kimura T,Ishizawa K,Mitsufuji T,Abe T,Nakazato Y,Yoshida K,Sasaki A,Araki N
Sun Dec 01 00:00:00 EST 2013
Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations.
Acta neuropathologica
Mackenzie IR,Arzberger T,Kremmer E,Troost D,Lorenzl S,Mori K,Weng SM,Haass C,Kretzschmar HA,Edbauer D,Neumann M
Sun Dec 01 00:00:00 EST 2013
Accumulation of phosphorylated TDP-43 in the CNS of a patient with Cockayne syndrome.
Neuropathology : official journal of the Japanese Society of Neuropathology
Sakurai A,Makioka K,Fukuda T,Takatama M,Okamoto K
Sun Dec 01 00:00:00 EST 2013
A unique mouse model for investigating the properties of amyotrophic lateral sclerosis-associated protein TDP-43, by in utero electroporation.
Neuroscience research
Akamatsu M,Takuma H,Yamashita T,Okada T,Keino-Masu K,Ishii K,Kwak S,Masu M,Tamaoka A
Sun Dec 01 00:00:00 EST 2013
Altered astrocytic expression of TDP-43 does not influence motor neuron survival.
Experimental neurology
Haidet-Phillips AM,Gross SK,Williams T,Tuteja A,Sherman A,Ko M,Jeong YH,Wong PC,Maragakis NJ
Sun Dec 01 00:00:00 EST 2013
Valosin-containing protein immunoreactivity in tauopathies, synucleinopathies, polyglutamine diseases and intranuclear inclusion body disease.
Neuropathology : official journal of the Japanese Society of Neuropathology
Mori F,Tanji K,Toyoshima Y,Sasaki H,Yoshida M,Kakita A,Takahashi H,Wakabayashi K
Sun Dec 01 00:00:00 EST 2013
Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration.
Proceedings of the National Academy of Sciences of the United States of America
Lagier-Tourenne C,Baughn M,Rigo F,Sun S,Liu P,Li HR,Jiang J,Watt AT,Chun S,Katz M,Qiu J,Sun Y,Ling SC,Zhu Q,Polymenidou M,Drenner K,Artates JW,McAlonis-Downes M,Markmiller S,Hutt KR,Pizzo DP,Cady J,Harms MB,Baloh RH,Vandenberg SR,Yeo GW,Fu XD,Bennett CF,Cleveland DW,Ravits J
Tue Nov 19 00:00:00 EST 2013
RNA binding mediates neurotoxicity in the transgenic Drosophila model of TDP-43 proteinopathy.
Human molecular genetics
Ihara R,Matsukawa K,Nagata Y,Kunugi H,Tsuji S,Chihara T,Kuranaga E,Miura M,Wakabayashi T,Hashimoto T,Iwatsubo T
Fri Nov 15 00:00:00 EST 2013
RNA binding mediates neurotoxicity in the transgenic Drosophila model of TDP-43 proteinopathy.
Human molecular genetics
Ihara R,Matsukawa K,Nagata Y,Kunugi H,Tsuji S,Chihara T,Kuranaga E,Miura M,Wakabayashi T,Hashimoto T,Iwatsubo T
Fri Nov 15 00:00:00 EST 2013
Activation of transforming growth factor-β/Smad signaling reduces aggregate formation of mislocalized TAR DNA-binding protein-43.
Neuro-degenerative diseases
Nakamura M,Kaneko S,Ito H,Jiang S,Fujita K,Wate R,Nakano S,Fujisawa J,Kusaka H
Wed Nov 13 00:00:00 EST 2013
Activation of transforming growth factor-β/Smad signaling reduces aggregate formation of mislocalized TAR DNA-binding protein-43.
Neuro-degenerative diseases
Nakamura M,Kaneko S,Ito H,Jiang S,Fujita K,Wate R,Nakano S,Fujisawa J,Kusaka H
Wed Nov 13 00:00:00 EST 2013
Quantitative neurofibrillary tangle density and brain volumetric MRI analyses in Alzheimer's disease presenting as logopenic progressive aphasia.
Brain and language
Josephs KA,Dickson DW,Murray ME,Senjem ML,Parisi JE,Petersen RC,Jack CR,Whitwell JL
Fri Nov 01 00:00:00 EDT 2013
Rescue of amyotrophic lateral sclerosis phenotype in a mouse model by intravenous AAV9-ADAR2 delivery to motor neurons.
EMBO molecular medicine
Yamashita T,Chai HL,Teramoto S,Tsuji S,Shimazaki K,Muramatsu S,Kwak S
Fri Nov 01 00:00:00 EDT 2013
Amyotrophic lateral sclerosis: an update on recent genetic insights.
Journal of neurology
Iguchi Y,Katsuno M,Ikenaka K,Ishigaki S,Sobue G
Fri Nov 01 00:00:00 EDT 2013
Targeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansion.
Science translational medicine
Sareen D,O'Rourke JG,Meera P,Muhammad AK,Grant S,Simpkinson M,Bell S,Carmona S,Ornelas L,Sahabian A,Gendron T,Petrucelli L,Baughn M,Ravits J,Harms MB,Rigo F,Bennett CF,Otis TS,Svendsen CN,Baloh RH
Wed Oct 23 00:00:00 EDT 2013
Characterization of the distal polyadenylation site of the ß-adducin (Add2) pre-mRNA.
PloS one
Costessi L,Porro F,Iaconcig A,Nedeljkovic M,Muro AF
Thu Oct 17 00:00:00 EDT 2013
RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention.
Neuron
Donnelly CJ,Zhang PW,Pham JT,Haeusler AR,Heusler AR,Mistry NA,Vidensky S,Daley EL,Poth EM,Hoover B,Fines DM,Maragakis N,Tienari PJ,Petrucelli L,Traynor BJ,Wang J,Rigo F,Bennett CF,Blackshaw S,Sattler R,Rothstein JD
Wed Oct 16 00:00:00 EDT 2013
Decreased number of Gemini of coiled bodies and U12 snRNA level in amyotrophic lateral sclerosis.
Human molecular genetics
Ishihara T,Ariizumi Y,Shiga A,Kato T,Tan CF,Sato T,Miki Y,Yokoo M,Fujino T,Koyama A,Yokoseki A,Nishizawa M,Kakita A,Takahashi H,Onodera O
Tue Oct 15 00:00:00 EDT 2013
Frontotemporal lobar degeneration: diversity of FTLD lesions.
Revue neurologique
Seilhean D,Bielle F,Plu I,Duyckaerts C
Tue Oct 01 00:00:00 EDT 2013
Non-targeted identification of prions and amyloid-forming proteins from yeast and mammalian cells.
The Journal of biological chemistry
Kryndushkin D,Pripuzova N,Burnett BG,Shewmaker F
Fri Sep 20 00:00:00 EDT 2013
Identification of genetic modifiers of TDP-43 neurotoxicity in Drosophila.
PloS one
Zhan L,Hanson KA,Kim SH,Tare A,Tibbetts RS
Tue Sep 10 00:00:00 EDT 2013
Identification of genetic modifiers of TDP-43 neurotoxicity in Drosophila.
PloS one
Zhan L,Hanson KA,Kim SH,Tare A,Tibbetts RS
Tue Sep 10 00:00:00 EDT 2013
Mutation analysis and immunopathological studies of PFN1 in familial and sporadic amyotrophic lateral sclerosis.
Neurobiology of aging
Yang S,Fifita JA,Williams KL,Warraich ST,Pamphlett R,Nicholson GA,Blair IP
Sun Sep 01 00:00:00 EDT 2013
β-N-methylamino-l-alanine causes neurological and pathological phenotypes mimicking Amyotrophic Lateral Sclerosis (ALS): the first step towards an experimental model for sporadic ALS.
Environmental toxicology and pharmacology
de Munck E,Muñoz-Sáez E,Miguel BG,Solas MT,Ojeda I,Martínez A,Gil C,Arahuetes RM
Sun Sep 01 00:00:00 EDT 2013
Sporadic ALS with compound heterozygous mutations in the SQSTM1 gene.
Acta neuropathologica
Shimizu H,Toyoshima Y,Shiga A,Yokoseki A,Arakawa K,Sekine Y,Shimohata T,Ikeuchi T,Nishizawa M,Kakita A,Onodera O,Takahashi H
Sun Sep 01 00:00:00 EDT 2013
A cellular model for sporadic ALS using patient-derived induced pluripotent stem cells.
Molecular and cellular neurosciences
Burkhardt MF,Martinez FJ,Wright S,Ramos C,Volfson D,Mason M,Garnes J,Dang V,Lievers J,Shoukat-Mumtaz U,Martinez R,Gai H,Blake R,Vaisberg E,Grskovic M,Johnson C,Irion S,Bright J,Cooper B,Nguyen L,Griswold-Prenner I,Javaherian A
Sun Sep 01 00:00:00 EDT 2013
Familial dementia with frontotemporal features associated with M146V presenilin-1 mutation.
Brain pathology (Zurich, Switzerland)
Riudavets MA,Bartoloni L,Troncoso JC,Pletnikova O,St George-Hyslop P,Schultz M,Sevlever G,Allegri RF
Sun Sep 01 00:00:00 EDT 2013
Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons.
Acta neuropathologica
Almeida S,Gascon E,Tran H,Chou HJ,Gendron TF,Degroot S,Tapper AR,Sellier C,Charlet-Berguerand N,Karydas A,Seeley WW,Boxer AL,Petrucelli L,Miller BL,Gao FB
Sun Sep 01 00:00:00 EDT 2013
Patterns of striatal degeneration in frontotemporal dementia.
Alzheimer disease and associated disorders
Halabi C,Halabi A,Dean DL,Wang PN,Boxer AL,Trojanowski JQ,Dearmond SJ,Miller BL,Kramer JH,Seeley WW
Mon Aug 12 00:00:00 EDT 2013
Reducing TDP-43 aggregation does not prevent its cytotoxicity.
Acta neuropathologica communications
Liu R,Yang G,Nonaka T,Arai T,Jia W,Cynader MS
Fri Aug 09 00:00:00 EDT 2013
Reducing TDP-43 aggregation does not prevent its cytotoxicity.
Acta neuropathologica communications
Liu R,Yang G,Nonaka T,Arai T,Jia W,Cynader MS
Fri Aug 09 00:00:00 EDT 2013
The dual functions of the extreme N-terminus of TDP-43 in regulating its biological activity and inclusion formation.
Human molecular genetics
Zhang YJ,Caulfield T,Xu YF,Gendron TF,Hubbard J,Stetler C,Sasaguri H,Whitelaw EC,Cai S,Lee WC,Petrucelli L
Thu Aug 01 00:00:00 EDT 2013
Surgical pathology of epilepsy-associated non-neoplastic cerebral lesions: a brief introduction with special reference to hippocampal sclerosis and focal cortical dysplasia.
Neuropathology : official journal of the Japanese Society of Neuropathology
Miyata H,Hori T,Vinters HV
Thu Aug 01 00:00:00 EDT 2013
Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice.
Molecular neurobiology
Janssens J,Wils H,Kleinberger G,Joris G,Cuijt I,Ceuterick-de Groote C,Van Broeckhoven C,Kumar-Singh S
Thu Aug 01 00:00:00 EDT 2013
Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice.
Molecular neurobiology
Janssens J,Wils H,Kleinberger G,Joris G,Cuijt I,Ceuterick-de Groote C,Van Broeckhoven C,Kumar-Singh S
Thu Aug 01 00:00:00 EDT 2013
Lack of unique neuropathology in amyotrophic lateral sclerosis associated with p.K54E angiogenin (ANG) mutation.
Neuropathology and applied neurobiology
Kirby J,Highley JR,Cox L,Goodall EF,Hewitt C,Hartley JA,Hollinger HC,Fox M,Ince PG,McDermott CJ,Shaw PJ
Thu Aug 01 00:00:00 EDT 2013
Functional genomic screen of human stem cell differentiation reveals pathways involved in neurodevelopment and neurodegeneration.
Proceedings of the National Academy of Sciences of the United States of America
Zhang Y,Schulz VP,Reed BD,Wang Z,Pan X,Mariani J,Euskirchen G,Snyder MP,Vaccarino FM,Ivanova N,Weissman SM,Szekely AM
Tue Jul 23 00:00:00 EDT 2013
Classification of FTLD-TDP cases into pathological subtypes using antibodies against phosphorylated and non-phosphorylated TDP43.
Acta neuropathologica communications
Tan RH,Shepherd CE,Kril JJ,McCann H,McGeachie A,McGinley C,Affleck A,Halliday GM
Wed Jul 10 00:00:00 EDT 2013
The long non-coding RNA nuclear-enriched abundant transcript 1_2 induces paraspeckle formation in the motor neuron during the early phase of amyotrophic lateral sclerosis.
Molecular brain
Nishimoto Y,Nakagawa S,Hirose T,Okano HJ,Takao M,Shibata S,Suyama S,Kuwako K,Imai T,Murayama S,Suzuki N,Okano H
Mon Jul 08 00:00:00 EDT 2013
The long non-coding RNA nuclear-enriched abundant transcript 1_2 induces paraspeckle formation in the motor neuron during the early phase of amyotrophic lateral sclerosis.
Molecular brain
Nishimoto Y,Nakagawa S,Hirose T,Okano HJ,Takao M,Shibata S,Suyama S,Kuwako K,Imai T,Murayama S,Suzuki N,Okano H
Mon Jul 08 00:00:00 EDT 2013
The pathogenesis of cingulate atrophy in behavioral variant frontotemporal dementia and Alzheimer's disease.
Acta neuropathologica communications
Tan RH,Pok K,Wong S,Brooks D,Halliday GM,Kril JJ
Fri Jul 05 00:00:00 EDT 2013
Neuron enriched nuclear proteome isolated from human brain.
Journal of proteome research
Dammer EB,Duong DM,Diner I,Gearing M,Feng Y,Lah JJ,Levey AI,Seyfried NT
Fri Jul 05 00:00:00 EDT 2013
Pharmacological reduction of ER stress protects against TDP-43 neuronal toxicity in vivo.
Neurobiology of disease
Vaccaro A,Patten SA,Aggad D,Julien C,Maios C,Kabashi E,Drapeau P,Parker JA
Mon Jul 01 00:00:00 EDT 2013
Selective forelimb impairment in rats expressing a pathological TDP-43 25 kDa C-terminal fragment to mimic amyotrophic lateral sclerosis.
Molecular therapy : the journal of the American Society of Gene Therapy
Dayton RD,Gitcho MA,Orchard EA,Wilson JD,Wang DB,Cain CD,Johnson JA,Zhang YJ,Petrucelli L,Mathis JM,Klein RL
Mon Jul 01 00:00:00 EDT 2013
Robust cytoplasmic accumulation of phosphorylated TDP-43 in transgenic models of tauopathy.
Acta neuropathologica
Clippinger AK,D'Alton S,Lin WL,Gendron TF,Howard J,Borchelt DR,Cannon A,Carlomagno Y,Chakrabarty P,Cook C,Golde TE,Levites Y,Ranum L,Schultheis PJ,Xu G,Petrucelli L,Sahara N,Dickson DW,Giasson B,Lewis J
Mon Jul 01 00:00:00 EDT 2013
Conserved acidic amino acid residues in a second RNA recognition motif regulate assembly and function of TDP-43.
PloS one
Shodai A,Ido A,Fujiwara N,Ayaki T,Morimura T,Oono M,Uchida T,Takahashi R,Ito H,Urushitani M
Thu Jun 27 00:00:00 EDT 2013
Conserved acidic amino acid residues in a second RNA recognition motif regulate assembly and function of TDP-43.
PloS one
Shodai A,Ido A,Fujiwara N,Ayaki T,Morimura T,Oono M,Uchida T,Takahashi R,Ito H,Urushitani M
Thu Jun 27 00:00:00 EDT 2013
Eukaryotic stress granules are cleared by autophagy and Cdc48/VCP function.
Cell
Buchan JR,Kolaitis RM,Taylor JP,Parker R
Thu Jun 20 00:00:00 EDT 2013
Tardbpl splicing rescues motor neuron and axonal development in a mutant tardbp zebrafish.
Human molecular genetics
Hewamadduma CA,Grierson AJ,Ma TP,Pan L,Moens CB,Ingham PW,Ramesh T,Shaw PJ
Sat Jun 15 00:00:00 EDT 2013
A role for calpain-dependent cleavage of TDP-43 in amyotrophic lateral sclerosis pathology.
Nature communications
Yamashita T,Hideyama T,Hachiga K,Teramoto S,Takano J,Iwata N,Saido TC,Kwak S
Tue Jun 11 00:00:00 EDT 2013
A role for calpain-dependent cleavage of TDP-43 in amyotrophic lateral sclerosis pathology.
Nature communications
Yamashita T,Hideyama T,Hachiga K,Teramoto S,Takano J,Iwata N,Saido TC,Kwak S
Tue Jun 11 00:00:00 EDT 2013
Comment on "Drug screening for ALS using patient-specific induced pluripotent stem cells".
Science translational medicine
Bilican B,Serio A,Barmada SJ,Nishimura AL,Sullivan GJ,Carrasco M,Phatnani HP,Puddifoot CA,Story D,Fletcher J,Park IH,Friedman BA,Daley GQ,Wyllie DJ,Hardingham GE,Wilmut I,Finkbeiner S,Maniatis T,Shaw CE,Chandran S
Wed Jun 05 00:00:00 EDT 2013
Comment on "Drug screening for ALS using patient-specific induced pluripotent stem cells".
Science translational medicine
Bilican B,Serio A,Barmada SJ,Nishimura AL,Sullivan GJ,Carrasco M,Phatnani HP,Puddifoot CA,Story D,Fletcher J,Park IH,Friedman BA,Daley GQ,Wyllie DJ,Hardingham GE,Wilmut I,Finkbeiner S,Maniatis T,Shaw CE,Chandran S
Wed Jun 05 00:00:00 EDT 2013
Laminar distribution of the pathological changes in sporadic frontotemporal lobar degeneration with transactive response (TAR) DNA-binding protein of 43 kDa (TDP-43) proteinopathy: a quantitative study using polynomial curve fitting.
Neuropathology and applied neurobiology
Armstrong RA,Hamilton RL,Mackenzie IR,Hedreen J,Cairns NJ
Sat Jun 01 00:00:00 EDT 2013
Progranulin mutations as risk factors for Alzheimer disease.
JAMA neurology
Perry DC,Lehmann M,Yokoyama JS,Karydas A,Lee JJ,Coppola G,Grinberg LT,Geschwind D,Seeley WW,Miller BL,Rosen H,Rabinovici G
Sat Jun 01 00:00:00 EDT 2013
Ubiquilin-2 (UBQLN2) binds with high affinity to the C-terminal region of TDP-43 and modulates TDP-43 levels in H4 cells: characterization of inhibition by nucleic acids and 4-aminoquinolines.
Biochimica et biophysica acta
Cassel JA,Reitz AB
Sat Jun 01 00:00:00 EDT 2013
α-Synuclein coaggregation in familial amyotrophic lateral sclerosis with SOD1 gene mutation.
Human pathology
Takei Y,Oguchi K,Koshihara H,Hineno A,Nakamura A,Ohara S
Sat Jun 01 00:00:00 EDT 2013
Optineurin is potentially associated with TDP-43 and involved in the pathogenesis of inclusion body myositis.
Neuropathology and applied neurobiology
Yamashita S,Kimura E,Tawara N,Sakaguchi H,Nakama T,Maeda Y,Hirano T,Uchino M,Ando Y
Sat Jun 01 00:00:00 EDT 2013
Aberrant assembly of RNA recognition motif 1 links to pathogenic conversion of TAR DNA-binding protein of 43 kDa (TDP-43).
The Journal of biological chemistry
Shodai A,Morimura T,Ido A,Uchida T,Ayaki T,Takahashi R,Kitazawa S,Suzuki S,Shirouzu M,Kigawa T,Muto Y,Yokoyama S,Takahashi R,Kitahara R,Ito H,Fujiwara N,Urushitani M
Fri May 24 00:00:00 EDT 2013
Extensive aggregation of α-synuclein and tau in juvenile-onset neuroaxonal dystrophy: an autopsied individual with a novel mutation in the PLA2G6 gene-splicing site.
Acta neuropathologica communications
Riku Y,Ikeuchi T,Yoshino H,Mimuro M,Mano K,Goto Y,Hattori N,Sobue G,Yoshida M
Thu May 09 00:00:00 EDT 2013
Expanded GGGGCC repeat RNA associated with amyotrophic lateral sclerosis and frontotemporal dementia causes neurodegeneration.
Proceedings of the National Academy of Sciences of the United States of America
Xu Z,Poidevin M,Li X,Li Y,Shu L,Nelson DL,Li H,Hales CM,Gearing M,Wingo TS,Jin P
Tue May 07 00:00:00 EDT 2013
Expanded GGGGCC repeat RNA associated with amyotrophic lateral sclerosis and frontotemporal dementia causes neurodegeneration.
Proceedings of the National Academy of Sciences of the United States of America
Xu Z,Poidevin M,Li X,Li Y,Shu L,Nelson DL,Li H,Hales CM,Gearing M,Wingo TS,Jin P
Tue May 07 00:00:00 EDT 2013
Loss of TDP-43 causes age-dependent progressive motor neuron degeneration.
Brain : a journal of neurology
Iguchi Y,Katsuno M,Niwa J,Takagi S,Ishigaki S,Ikenaka K,Kawai K,Watanabe H,Yamanaka K,Takahashi R,Misawa H,Sasaki S,Tanaka F,Sobue G
Wed May 01 00:00:00 EDT 2013
Loss of TDP-43 causes age-dependent progressive motor neuron degeneration.
Brain : a journal of neurology
Iguchi Y,Katsuno M,Niwa J,Takagi S,Ishigaki S,Ikenaka K,Kawai K,Watanabe H,Yamanaka K,Takahashi R,Misawa H,Sasaki S,Tanaka F,Sobue G
Wed May 01 00:00:00 EDT 2013
C9ORF72 expansion in a family with bipolar disorder.
Bipolar disorders
Meisler MH,Grant AE,Jones JM,Lenk GM,He F,Todd PK,Kamali M,Albin RL,Lieberman AP,Langenecker SA,McInnis MG
Wed May 01 00:00:00 EDT 2013
Japanese amyotrophic lateral sclerosis patients with GGGGCC hexanucleotide repeat expansion in C9ORF72.
Journal of neurology, neurosurgery, and psychiatry
Konno T,Shiga A,Tsujino A,Sugai A,Kato T,Kanai K,Yokoseki A,Eguchi H,Kuwabara S,Nishizawa M,Takahashi H,Onodera O
Mon Apr 01 00:00:00 EDT 2013
Welander distal myopathy caused by an ancient founder mutation in TIA1 associated with perturbed splicing.
Human mutation
Klar J,Sobol M,Melberg A,Mäbert K,Ameur A,Johansson AC,Feuk L,Entesarian M,Orlén H,Casar-Borota O,Dahl N
Mon Apr 01 00:00:00 EDT 2013
G51D α-synuclein mutation causes a novel parkinsonian-pyramidal syndrome.
Annals of neurology
Lesage S,Anheim M,Letournel F,Bousset L,Honoré A,Rozas N,Pieri L,Madiona K,Dürr A,Melki R,Verny C,Brice A
Mon Apr 01 00:00:00 EDT 2013
The truncated C-terminal RNA recognition motif of TDP-43 protein plays a key role in forming proteinaceous aggregates.
The Journal of biological chemistry
Wang YT,Kuo PH,Chiang CH,Liang JR,Chen YR,Wang S,Shen JC,Yuan HS
Fri Mar 29 00:00:00 EDT 2013
Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS.
Nature
Kim HJ,Kim NC,Wang YD,Scarborough EA,Moore J,Diaz Z,MacLea KS,Freibaum B,Li S,Molliex A,Kanagaraj AP,Carter R,Boylan KB,Wojtas AM,Rademakers R,Pinkus JL,Greenberg SA,Trojanowski JQ,Traynor BJ,Smith BN,Topp S,Gkazi AS,Miller J,Shaw CE,Kottlors M,Kirschner J,Pestronk A,Li YR,Ford AF,Gitler AD,Benatar M,King OD,Kimonis VE,Ross ED,Weihl CC,Shorter J,Taylor JP
Thu Mar 28 00:00:00 EDT 2013
Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS.
Nature
Kim HJ,Kim NC,Wang YD,Scarborough EA,Moore J,Diaz Z,MacLea KS,Freibaum B,Li S,Molliex A,Kanagaraj AP,Carter R,Boylan KB,Wojtas AM,Rademakers R,Pinkus JL,Greenberg SA,Trojanowski JQ,Traynor BJ,Smith BN,Topp S,Gkazi AS,Miller J,Shaw CE,Kottlors M,Kirschner J,Pestronk A,Li YR,Ford AF,Gitler AD,Benatar M,King OD,Kimonis VE,Ross ED,Weihl CC,Shorter J,Taylor JP
Thu Mar 28 00:00:00 EDT 2013
Coaggregation of RNA-binding proteins in a model of TDP-43 proteinopathy with selective RGG motif methylation and a role for RRM1 ubiquitination.
PloS one
Dammer EB,Fallini C,Gozal YM,Duong DM,Rossoll W,Xu P,Lah JJ,Levey AI,Peng J,Bassell GJ,Seyfried NT
Tue Mar 26 00:00:00 EDT 2013
Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain.
Scientific reports
Rogelj B,Easton LE,Bogu GK,Stanton LW,Rot G,Curk T,Zupan B,Sugimoto Y,Modic M,Haberman N,Tollervey J,Fujii R,Takumi T,Shaw CE,Ule J
Fri Mar 22 00:00:00 EDT 2013
Alteration of POLDIP3 splicing associated with loss of function of TDP-43 in tissues affected with ALS.
PloS one
Shiga A,Ishihara T,Miyashita A,Kuwabara M,Kato T,Watanabe N,Yamahira A,Kondo C,Yokoseki A,Takahashi M,Kuwano R,Kakita A,Nishizawa M,Takahashi H,Onodera O
Fri Mar 22 00:00:00 EDT 2013
Alteration of POLDIP3 splicing associated with loss of function of TDP-43 in tissues affected with ALS.
PloS one
Shiga A,Ishihara T,Miyashita A,Kuwabara M,Kato T,Watanabe N,Yamahira A,Kondo C,Yokoseki A,Takahashi M,Kuwano R,Kakita A,Nishizawa M,Takahashi H,Onodera O
Fri Mar 22 00:00:00 EDT 2013
Alteration of POLDIP3 splicing associated with loss of function of TDP-43 in tissues affected with ALS.
PloS one
Shiga A,Ishihara T,Miyashita A,Kuwabara M,Kato T,Watanabe N,Yamahira A,Kondo C,Yokoseki A,Takahashi M,Kuwano R,Kakita A,Nishizawa M,Takahashi H,Onodera O
Fri Mar 22 00:00:00 EDT 2013
Exome sequencing identifies titin mutations causing hereditary myopathy with early respiratory failure (HMERF) in families of diverse ethnic origins.
BMC neurology
Toro C,Olivé M,Dalakas MC,Sivakumar K,Bilbao JM,Tyndel F,Vidal N,Farrero E,Sambuughin N,Goldfarb LG
Wed Mar 20 00:00:00 EDT 2013
Astrocyte pathology and the absence of non-cell autonomy in an induced pluripotent stem cell model of TDP-43 proteinopathy.
Proceedings of the National Academy of Sciences of the United States of America
Serio A,Bilican B,Barmada SJ,Ando DM,Zhao C,Siller R,Burr K,Haghi G,Story D,Nishimura AL,Carrasco MA,Phatnani HP,Shum C,Wilmut I,Maniatis T,Shaw CE,Finkbeiner S,Chandran S
Tue Mar 19 00:00:00 EDT 2013
Astrocyte pathology and the absence of non-cell autonomy in an induced pluripotent stem cell model of TDP-43 proteinopathy.
Proceedings of the National Academy of Sciences of the United States of America
Serio A,Bilican B,Barmada SJ,Ando DM,Zhao C,Siller R,Burr K,Haghi G,Story D,Nishimura AL,Carrasco MA,Phatnani HP,Shum C,Wilmut I,Maniatis T,Shaw CE,Finkbeiner S,Chandran S
Tue Mar 19 00:00:00 EDT 2013
Luminescent conjugated oligothiophenes for sensitive fluorescent assignment of protein inclusion bodies.
Chembiochem : a European journal of chemical biology
Klingstedt T,Blechschmidt C,Nogalska A,Prokop S,Häggqvist B,Danielsson O,Engel WK,Askanas V,Heppner FL,Nilsson KP
Mon Mar 18 00:00:00 EDT 2013
The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS.
Science (New York, N.Y.)
Mori K,Weng SM,Arzberger T,May S,Rentzsch K,Kremmer E,Schmid B,Kretzschmar HA,Cruts M,Van Broeckhoven C,Haass C,Edbauer D
Fri Mar 15 00:00:00 EDT 2013
DNAJB6 myopathy in an Asian cohort and cytoplasmic/nuclear inclusions.
Neuromuscular disorders : NMD
Sato T,Hayashi YK,Oya Y,Kondo T,Sugie K,Kaneda D,Houzen H,Yabe I,Sasaki H,Noguchi S,Nonaka I,Osawa M,Nishino I
Fri Mar 01 00:00:00 EST 2013
Hippocampal sclerosis dementia: An amnesic variant of frontotemporal degeneration.
Dementia & neuropsychologia
Onyike CU,Pletnikova O,Sloane KL,Sullivan C,Troncoso JC,Rabins PV
Fri Mar 01 00:00:00 EST 2013
Making the diagnosis of frontotemporal lobar degeneration.
Archives of pathology & laboratory medicine
Bigio EH
Fri Mar 01 00:00:00 EST 2013
Identification of in vivo, conserved, TAF15 RNA binding sites reveals the impact of TAF15 on the neuronal transcriptome.
Cell reports
Ibrahim F,Maragkakis M,Alexiou P,Maronski MA,Dichter MA,Mourelatos Z
Thu Feb 21 00:00:00 EST 2013
The frontotemporal lobar degeneration risk factor, TMEM106B, regulates lysosomal morphology and function.
Human molecular genetics
Brady OA,Zheng Y,Murphy K,Huang M,Hu F
Fri Feb 15 00:00:00 EST 2013
The frontotemporal lobar degeneration risk factor, TMEM106B, regulates lysosomal morphology and function.
Human molecular genetics
Brady OA,Zheng Y,Murphy K,Huang M,Hu F
Fri Feb 15 00:00:00 EST 2013
Reduction of polyglutamine toxicity by TDP-43, FUS and progranulin in Huntington's disease models.
Human molecular genetics
Tauffenberger A,Chitramuthu BP,Bateman A,Bennett HP,Parker JA
Fri Feb 15 00:00:00 EST 2013
Parkin ubiquitinates Tar-DNA binding protein-43 (TDP-43) and promotes its cytosolic accumulation via interaction with histone deacetylase 6 (HDAC6).
The Journal of biological chemistry
Hebron ML,Lonskaya I,Sharpe K,Weerasinghe PP,Algarzae NK,Shekoyan AR,Moussa CE
Fri Feb 08 00:00:00 EST 2013
Parkin ubiquitinates Tar-DNA binding protein-43 (TDP-43) and promotes its cytosolic accumulation via interaction with histone deacetylase 6 (HDAC6).
The Journal of biological chemistry
Hebron ML,Lonskaya I,Sharpe K,Weerasinghe PP,Algarzae NK,Shekoyan AR,Moussa CE
Fri Feb 08 00:00:00 EST 2013
Parkin ubiquitinates Tar-DNA binding protein-43 (TDP-43) and promotes its cytosolic accumulation via interaction with histone deacetylase 6 (HDAC6).
The Journal of biological chemistry
Hebron ML,Lonskaya I,Sharpe K,Weerasinghe PP,Algarzae NK,Shekoyan AR,Moussa CE
Fri Feb 08 00:00:00 EST 2013
Nuclear carrier and RNA-binding proteins in frontotemporal lobar degeneration associated with fused in sarcoma (FUS) pathological changes.
Neuropathology and applied neurobiology
Davidson YS,Robinson AC,Hu Q,Mishra M,Baborie A,Jaros E,Perry RH,Cairns NJ,Richardson A,Gerhard A,Neary D,Snowden JS,Bigio EH,Mann DM
Fri Feb 01 00:00:00 EST 2013
Degeneration of serotonergic neurons in amyotrophic lateral sclerosis: a link to spasticity.
Brain : a journal of neurology
Dentel C,Palamiuc L,Henriques A,Lannes B,Spreux-Varoquaux O,Gutknecht L,René F,Echaniz-Laguna A,Gonzalez de Aguilar JL,Lesch KP,Meininger V,Loeffler JP,Dupuis L
Fri Feb 01 00:00:00 EST 2013
Regionally different immunoreactivity for Smurf2 and pSmad2/3 in TDP-43-positive inclusions of amyotrophic lateral sclerosis.
Neuropathology and applied neurobiology
Nakamura M,Kaneko S,Wate R,Asayama S,Nakamura Y,Fujita K,Ito H,Kusaka H
Fri Feb 01 00:00:00 EST 2013
Premature death of TDP-43 (A315T) transgenic mice due to gastrointestinal complications prior to development of full neurological symptoms of amyotrophic lateral sclerosis.
International journal of experimental pathology
Esmaeili MA,Panahi M,Yadav S,Hennings L,Kiaei M
Fri Feb 01 00:00:00 EST 2013
Premature death of TDP-43 (A315T) transgenic mice due to gastrointestinal complications prior to development of full neurological symptoms of amyotrophic lateral sclerosis.
International journal of experimental pathology
Esmaeili MA,Panahi M,Yadav S,Hennings L,Kiaei M
Fri Feb 01 00:00:00 EST 2013
Spliceosome integrity is defective in the motor neuron diseases ALS and SMA.
EMBO molecular medicine
Tsuiji H,Iguchi Y,Furuya A,Kataoka A,Hatsuta H,Atsuta N,Tanaka F,Hashizume Y,Akatsu H,Murayama S,Sobue G,Yamanaka K
Fri Feb 01 00:00:00 EST 2013
Spliceosome integrity is defective in the motor neuron diseases ALS and SMA.
EMBO molecular medicine
Tsuiji H,Iguchi Y,Furuya A,Kataoka A,Hatsuta H,Atsuta N,Tanaka F,Hashizume Y,Akatsu H,Murayama S,Sobue G,Yamanaka K
Fri Feb 01 00:00:00 EST 2013
Oligodendrocyte dysfunction in the pathogenesis of amyotrophic lateral sclerosis.
Brain : a journal of neurology
Philips T,Bento-Abreu A,Nonneman A,Haeck W,Staats K,Geelen V,Hersmus N,Küsters B,Van Den Bosch L,Van Damme P,Richardson WD,Robberecht W
Fri Feb 01 00:00:00 EST 2013
Pantothenate kinase-associated neurodegeneration is not a synucleinopathy.
Neuropathology and applied neurobiology
Li A,Paudel R,Johnson R,Courtney R,Lees AJ,Holton JL,Hardy J,Revesz T,Houlden H
Fri Feb 01 00:00:00 EST 2013
Corticospinal tract degeneration associated with TDP-43 type C pathology and semantic dementia.
Brain : a journal of neurology
Josephs KA,Whitwell JL,Murray ME,Parisi JE,Graff-Radford NR,Knopman DS,Boeve BF,Senjem ML,Rademakers R,Jack CR,Petersen RC,Dickson DW
Fri Feb 01 00:00:00 EST 2013
Atypical FTLD-FUS associated with ALS-TDP: a case report.
Neuropathology : official journal of the Japanese Society of Neuropathology
Kobayashi Z,Arai T,Yokota O,Tsuchiya K,Hosokawa M,Oshima K,Niizato K,Akiyama H,Mizusawa H
Fri Feb 01 00:00:00 EST 2013
Structural and molecular insights into the mechanism of action of human angiogenin-ALS variants in neurons.
Nature communications
Thiyagarajan N,Ferguson R,Subramanian V,Acharya KR
Fri Feb 01 00:00:00 EST 2013
A fatal neuromuscular disease in an adult patient after poliomyelitis in early childhood: consideration of the pathology of post-polio syndrome.
Neuropathology : official journal of the Japanese Society of Neuropathology
Kosaka T,Kuroha Y,Tada M,Hasegawa A,Tani T,Matsubara N,Koike R,Toyoshima Y,Takahashi H
Fri Feb 01 00:00:00 EST 2013
Co-occurrence of TDP-43 mislocalization with reduced activity of an RNA editing enzyme, ADAR2, in aged mouse motor neurons.
PloS one
Hideyama T,Teramoto S,Hachiga K,Yamashita T,Kwak S
Wed Jan 16 00:00:00 EST 2013
Glucocorticoids exacerbate cognitive deficits in TDP-25 transgenic mice via a glutathione-mediated mechanism: implications for aging, stress and TDP-43 proteinopathies.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Caccamo A,Medina DX,Oddo S
Wed Jan 16 00:00:00 EST 2013
New NBIA subtype: genetic, clinical, pathologic, and radiographic features of MPAN.
Neurology
Hogarth P,Gregory A,Kruer MC,Sanford L,Wagoner W,Natowicz MR,Egel RT,Subramony SH,Goldman JG,Berry-Kravis E,Foulds NC,Hammans SR,Desguerre I,Rodriguez D,Wilson C,Diedrich A,Green S,Tran H,Reese L,Woltjer RL,Hayflick SJ
Tue Jan 15 00:00:00 EST 2013
Involvement of endoplasmic reticulum stress in tauopathy.
Biochemical and biophysical research communications
Sakagami Y,Kudo T,Tanimukai H,Kanayama D,Omi T,Horiguchi K,Okochi M,Imaizumi K,Takeda M
Fri Jan 11 00:00:00 EST 2013
Inhibition of TDP-43 accumulation by bis(thiosemicarbazonato)-copper complexes.
PloS one
Parker SJ,Meyerowitz J,James JL,Liddell JR,Nonaka T,Hasegawa M,Kanninen KM,Lim S,Paterson BM,Donnelly PS,Crouch PJ,White AR
Thu Jan 10 00:00:00 EST 2013
Inhibition of TDP-43 accumulation by bis(thiosemicarbazonato)-copper complexes.
PloS one
Parker SJ,Meyerowitz J,James JL,Liddell JR,Nonaka T,Hasegawa M,Kanninen KM,Lim S,Paterson BM,Donnelly PS,Crouch PJ,White AR
Thu Jan 10 00:00:00 EST 2013
Methylene blue protects against TDP-43 and FUS neuronal toxicity in C. elegans and D. rerio.
PloS one
Vaccaro A,Patten SA,Ciura S,Maios C,Therrien M,Drapeau P,Kabashi E,Parker JA
Thu Jan 10 00:00:00 EST 2013
Widespread aggregation of mutant VAPB associated with ALS does not cause motor neuron degeneration or modulate mutant SOD1 aggregation and toxicity in mice.
Molecular neurodegeneration
Qiu L,Qiao T,Beers M,Tan W,Wang H,Yang B,Xu Z
Thu Jan 03 00:00:00 EST 2013
Expression of myogenic regulatory factors and myo-endothelial remodeling in sporadic inclusion body myositis.
Neuromuscular disorders : NMD
Wanschitz JV,Dubourg O,Lacene E,Fischer MB,Höftberger R,Budka H,Romero NB,Eymard B,Herson S,Butler-Browne GS,Voit T,Benveniste O
Tue Jan 01 00:00:00 EST 2013
African ancestry protects against Alzheimer's disease-related neuropathology.
Molecular psychiatry
Schlesinger D,Grinberg LT,Alba JG,Naslavsky MS,Licinio L,Farfel JM,Suemoto CK,de Lucena Ferretti RE,Leite RE,de Andrade MP,dos Santos AC,Brentani H,Pasqualucci CA,Nitrini R,Jacob-Filho W,Zatz M
Tue Jan 01 00:00:00 EST 2013
Essential tremor followed by progressive supranuclear palsy: postmortem reports of 11 patients.
Journal of neuropathology and experimental neurology
Louis ED,Babij R,Ma K,Cortés E,Vonsattel JP
Tue Jan 01 00:00:00 EST 2013
Familial ALS with FUS P525L mutation: two Japanese sisters with multiple systems involvement.
Journal of the neurological sciences
Mochizuki Y,Isozaki E,Takao M,Hashimoto T,Shibuya M,Arai M,Hosokawa M,Kawata A,Oyanagi K,Mihara B,Mizutani T
Sat Dec 15 00:00:00 EST 2012
Motor neuron-specific disruption of proteasomes, but not autophagy, replicates amyotrophic lateral sclerosis.
The Journal of biological chemistry
Tashiro Y,Urushitani M,Inoue H,Koike M,Uchiyama Y,Komatsu M,Tanaka K,Yamazaki M,Abe M,Misawa H,Sakimura K,Ito H,Takahashi R
Fri Dec 14 00:00:00 EST 2012
Differential expression of TAR DNA-binding protein (TDP-43) in the central nervous system of horses afflicted with equine motor neuron disease (EMND): a preliminary study of a potential pathologic marker.
Veterinary research communications
El-Assaad I,Di Bari JA,Yasuda K,Divers TJ,Summers BA,de Lahunta A,Mohammed H
Sat Dec 01 00:00:00 EST 2012
Differential expression of TAR DNA-binding protein (TDP-43) in the central nervous system of horses afflicted with equine motor neuron disease (EMND): a preliminary study of a potential pathologic marker.
Veterinary research communications
El-Assaad I,Di Bari JA,Yasuda K,Divers TJ,Summers BA,de Lahunta A,Mohammed H
Sat Dec 01 00:00:00 EST 2012
Association of UBQLN1 mutation with Brown-Vialetto-Van Laere syndrome but not typical ALS.
Neurobiology of disease
González-Pérez P,Lu Y,Chian RJ,Sapp PC,Tanzi RE,Bertram L,McKenna-Yasek D,Gao FB,Brown RH
Sat Dec 01 00:00:00 EST 2012
Association of UBQLN1 mutation with Brown-Vialetto-Van Laere syndrome but not typical ALS.
Neurobiology of disease
González-Pérez P,Lu Y,Chian RJ,Sapp PC,Tanzi RE,Bertram L,McKenna-Yasek D,Gao FB,Brown RH
Sat Dec 01 00:00:00 EST 2012
Mitochondrial dysfunction in human TDP-43 transfected NSC34 cell lines and the protective effect of dimethoxy curcumin.
Brain research bulletin
Lu J,Duan W,Guo Y,Jiang H,Li Z,Huang J,Hong K,Li C
Sat Dec 01 00:00:00 EST 2012
Mitochondrial dysfunction in human TDP-43 transfected NSC34 cell lines and the protective effect of dimethoxy curcumin.
Brain research bulletin
Lu J,Duan W,Guo Y,Jiang H,Li Z,Huang J,Hong K,Li C
Sat Dec 01 00:00:00 EST 2012
Genetic association of CR1 with Alzheimer's disease: a tentative disease mechanism.
Neurobiology of aging
Hazrati LN,Van Cauwenberghe C,Brooks PL,Brouwers N,Ghani M,Sato C,Cruts M,Sleegers K,St George-Hyslop P,Van Broeckhoven C,Rogaeva E
Sat Dec 01 00:00:00 EST 2012
Long non-coding RNA gadd7 interacts with TDP-43 and regulates Cdk6 mRNA decay.
The EMBO journal
Liu X,Li D,Zhang W,Guo M,Zhan Q
Wed Nov 28 00:00:00 EST 2012
Long non-coding RNA gadd7 interacts with TDP-43 and regulates Cdk6 mRNA decay.
The EMBO journal
Liu X,Li D,Zhang W,Guo M,Zhan Q
Wed Nov 28 00:00:00 EST 2012
An early onset progressive motor neuron disorder in Scyl1-deficient mice is associated with mislocalization of TDP-43.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Pelletier S,Gingras S,Howell S,Vogel P,Ihle JN
Wed Nov 21 00:00:00 EST 2012
An early onset progressive motor neuron disorder in Scyl1-deficient mice is associated with mislocalization of TDP-43.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Pelletier S,Gingras S,Howell S,Vogel P,Ihle JN
Wed Nov 21 00:00:00 EST 2012
Minocycline-associated rimmed vacuolar myopathy in a patient with rheumatoid arthritis.
BMC neurology
Bokuda K,Sugaya K,Tamura S,Miyamoto K,Matsubara S,Komori T
Wed Nov 21 00:00:00 EST 2012
Iron accumulation in deep cortical layers accounts for MRI signal abnormalities in ALS: correlating 7 tesla MRI and pathology.
PloS one
Kwan JY,Jeong SY,Van Gelderen P,Deng HX,Quezado MM,Danielian LE,Butman JA,Chen L,Bayat E,Russell J,Siddique T,Duyn JH,Rouault TA,Floeter MK
Mon Nov 19 00:00:00 EST 2012
High-content RNAi screening identifies the Type 1 inositol triphosphate receptor as a modifier of TDP-43 localization and neurotoxicity.
Human molecular genetics
Kim SH,Zhan L,Hanson KA,Tibbetts RS
Thu Nov 15 00:00:00 EST 2012
High-content RNAi screening identifies the Type 1 inositol triphosphate receptor as a modifier of TDP-43 localization and neurotoxicity.
Human molecular genetics
Kim SH,Zhan L,Hanson KA,Tibbetts RS
Thu Nov 15 00:00:00 EST 2012
Characterization of staufen1 ribonucleoproteins by mass spectrometry and biochemical analyses reveal the presence of diverse host proteins associated with human immunodeficiency virus type 1.
Frontiers in microbiology
Milev MP,Ravichandran M,Khan MF,Schriemer DC,Mouland AJ
Tue Nov 06 00:00:00 EST 2012
Characterization of staufen1 ribonucleoproteins by mass spectrometry and biochemical analyses reveal the presence of diverse host proteins associated with human immunodeficiency virus type 1.
Frontiers in microbiology
Milev MP,Ravichandran M,Khan MF,Schriemer DC,Mouland AJ
Tue Nov 06 00:00:00 EST 2012
Lack of miRNA Misregulation at Early Pathological Stages in Drosophila Neurodegenerative Disease Models.
Frontiers in genetics
Reinhardt A,Feuillette S,Cassar M,Callens C,Thomassin H,Birman S,Lecourtois M,Antoniewski C,Tricoire H
Fri Nov 02 00:00:00 EDT 2012
Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs.
Nature neuroscience
Lagier-Tourenne C,Polymenidou M,Hutt KR,Vu AQ,Baughn M,Huelga SC,Clutario KM,Ling SC,Liang TY,Mazur C,Wancewicz E,Kim AS,Watt A,Freier S,Hicks GG,Donohue JP,Shiue L,Bennett CF,Ravits J,Cleveland DW,Yeo GW
Thu Nov 01 00:00:00 EDT 2012
Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs.
Nature neuroscience
Lagier-Tourenne C,Polymenidou M,Hutt KR,Vu AQ,Baughn M,Huelga SC,Clutario KM,Ling SC,Liang TY,Mazur C,Wancewicz E,Kim AS,Watt A,Freier S,Hicks GG,Donohue JP,Shiue L,Bennett CF,Ravits J,Cleveland DW,Yeo GW
Thu Nov 01 00:00:00 EDT 2012
The RNA-binding motif 45 (RBM45) protein accumulates in inclusion bodies in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) patients.
Acta neuropathologica
Collins M,Riascos D,Kovalik T,An J,Krupa K,Krupa K,Hood BL,Conrads TP,Renton AE,Traynor BJ,Bowser R
Thu Nov 01 00:00:00 EDT 2012
The RNA-binding motif 45 (RBM45) protein accumulates in inclusion bodies in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) patients.
Acta neuropathologica
Collins M,Riascos D,Kovalik T,An J,Krupa K,Krupa K,Hood BL,Conrads TP,Renton AE,Traynor BJ,Bowser R
Thu Nov 01 00:00:00 EDT 2012
Convergent transcription induces transcriptional gene silencing in fission yeast and mammalian cells.
Nature structural & molecular biology
Gullerova M,Proudfoot NJ
Thu Nov 01 00:00:00 EDT 2012
Molecular analysis and biochemical classification of TDP-43 proteinopathy.
Brain : a journal of neurology
Tsuji H,Arai T,Kametani F,Nonaka T,Yamashita M,Suzukake M,Hosokawa M,Yoshida M,Hatsuta H,Takao M,Saito Y,Murayama S,Akiyama H,Hasegawa M,Mann DM,Tamaoka A
Thu Nov 01 00:00:00 EDT 2012
Entorhinal cortical neurons are the primary targets of FUS mislocalization and ubiquitin aggregation in FUS transgenic rats.
Human molecular genetics
Huang C,Tong J,Bi F,Wu Q,Huang B,Zhou H,Xia XG
Thu Nov 01 00:00:00 EDT 2012
Coexistence of Huntington's disease and amyotrophic lateral sclerosis: a clinicopathologic study.
Acta neuropathologica
Tada M,Coon EA,Osmand AP,Kirby PA,Martin W,Wieler M,Shiga A,Shirasaki H,Tada M,Makifuchi T,Yamada M,Kakita A,Nishizawa M,Takahashi H,Paulson HL
Thu Nov 01 00:00:00 EDT 2012
Induced pluripotent stem cell models of progranulin-deficient frontotemporal dementia uncover specific reversible neuronal defects.
Cell reports
Almeida S,Zhang Z,Coppola G,Mao W,Futai K,Karydas A,Geschwind MD,Tartaglia MC,Gao F,Gianni D,Sena-Esteves M,Geschwind DH,Miller BL,Farese RV,Gao FB
Thu Oct 25 00:00:00 EDT 2012
FUS-SMN protein interactions link the motor neuron diseases ALS and SMA.
Cell reports
Yamazaki T,Chen S,Yu Y,Yan B,Haertlein TC,Carrasco MA,Tapia JC,Zhai B,Das R,Lalancette-Hebert M,Sharma A,Chandran S,Sullivan G,Nishimura AL,Shaw CE,Gygi SP,Shneider NA,Maniatis T,Reed R
Thu Oct 25 00:00:00 EDT 2012
FUS-SMN protein interactions link the motor neuron diseases ALS and SMA.
Cell reports
Yamazaki T,Chen S,Yu Y,Yan B,Haertlein TC,Carrasco MA,Tapia JC,Zhai B,Das R,Lalancette-Hebert M,Sharma A,Chandran S,Sullivan G,Nishimura AL,Shaw CE,Gygi SP,Shneider NA,Maniatis T,Reed R
Thu Oct 25 00:00:00 EDT 2012
FUS-SMN protein interactions link the motor neuron diseases ALS and SMA.
Cell reports
Yamazaki T,Chen S,Yu Y,Yan B,Haertlein TC,Carrasco MA,Tapia JC,Zhai B,Das R,Lalancette-Hebert M,Sharma A,Chandran S,Sullivan G,Nishimura AL,Shaw CE,Gygi SP,Shneider NA,Maniatis T,Reed R
Thu Oct 25 00:00:00 EDT 2012
Endogenous TDP-43, but not FUS, contributes to stress granule assembly via G3BP.
Molecular neurodegeneration
Aulas A,Stabile S,Vande Velde C
Wed Oct 24 00:00:00 EDT 2012
Endogenous TDP-43, but not FUS, contributes to stress granule assembly via G3BP.
Molecular neurodegeneration
Aulas A,Stabile S,Vande Velde C
Wed Oct 24 00:00:00 EDT 2012
Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesis.
Molecular neurodegeneration
Verbeeck C,Deng Q,Dejesus-Hernandez M,Taylor G,Ceballos-Diaz C,Kocerha J,Golde T,Das P,Rademakers R,Dickson DW,Kukar T
Wed Oct 10 00:00:00 EDT 2012
Endosomal sorting related protein CHMP2B is localized in Lewy bodies and glial cytoplasmic inclusions in α-synucleinopathy.
Neuroscience letters
Tanikawa S,Mori F,Tanji K,Kakita A,Takahashi H,Wakabayashi K
Wed Oct 03 00:00:00 EDT 2012
In vivo and post-mortem memory circuit integrity in frontotemporal dementia and Alzheimer's disease.
Brain : a journal of neurology
Hornberger M,Wong S,Tan R,Irish M,Piguet O,Kril J,Hodges JR,Halliday G
Mon Oct 01 00:00:00 EDT 2012
An MND/ALS phenotype associated with C9orf72 repeat expansion: abundant p62-positive, TDP-43-negative inclusions in cerebral cortex, hippocampus and cerebellum but without associated cognitive decline.
Neuropathology : official journal of the Japanese Society of Neuropathology
Troakes C,Maekawa S,Wijesekera L,Rogelj B,Siklós L,Bell C,Smith B,Newhouse S,Vance C,Johnson L,Hortobágyi T,Shatunov A,Al-Chalabi A,Leigh N,Shaw CE,King A,Al-Sarraj S
Mon Oct 01 00:00:00 EDT 2012
Benign hereditary chorea 2: pathological findings in an autopsy case.
Neuropathology : official journal of the Japanese Society of Neuropathology
Yoshida Y,Nunomura J,Shimohata T,Nanjo H,Miyata H
Mon Oct 01 00:00:00 EDT 2012
Glucose delays age-dependent proteotoxicity.
Aging cell
Tauffenberger A,Vaccaro A,Aulas A,Vande Velde C,Parker JA
Mon Oct 01 00:00:00 EDT 2012
UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis.
Neurobiology of aging
Williams KL,Warraich ST,Yang S,Solski JA,Fernando R,Rouleau GA,Nicholson GA,Blair IP
Mon Oct 01 00:00:00 EDT 2012
TDP-43 identified from a genome wide RNAi screen for SOD1 regulators.
PloS one
Somalinga BR,Day CE,Wei S,Roth MG,Thomas PJ
Mon Sep 17 00:00:00 EDT 2012
Clinical, neuropathological, and genetic characteristics of the novel IVS9+1delG GRN mutation in a patient with frontotemporal dementia.
Journal of Alzheimer's disease : JAD
Taipa R,Tuna A,Damásio J,Pinto PS,Cavaco S,Pereira S,Milterberger-Miltenyi G,Galimberti D,Melo-Pires M
Mon Sep 10 00:00:00 EDT 2012
Aberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosis.
PloS one
Pokrishevsky E,Grad LI,Yousefi M,Wang J,Mackenzie IR,Cashman NR
Tue Sep 04 00:00:00 EDT 2012
Aberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosis.
PloS one
Pokrishevsky E,Grad LI,Yousefi M,Wang J,Mackenzie IR,Cashman NR
Tue Sep 04 00:00:00 EDT 2012
A novel TARDBP insertion/deletion mutation in the flail arm variant of amyotrophic lateral sclerosis.
Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
Solski JA,Yang S,Nicholson GA,Luquin N,Williams KL,Fernando R,Pamphlett R,Blair IP
Sat Sep 01 00:00:00 EDT 2012
FUS/TLS-immunoreactive neuronal and glial cell inclusions increase with disease duration in familial amyotrophic lateral sclerosis with an R521C FUS/TLS mutation.
Journal of neuropathology and experimental neurology
Suzuki N,Kato S,Kato M,Warita H,Mizuno H,Kato M,Shimakura N,Akiyama H,Kobayashi Z,Konno H,Aoki M
Sat Sep 01 00:00:00 EDT 2012
TDP-43 plasma levels are higher in amyotrophic lateral sclerosis.
Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
Verstraete E,Kuiperij HB,van Blitterswijk MM,Veldink JH,Schelhaas HJ,van den Berg LH,Verbeek MM
Sat Sep 01 00:00:00 EDT 2012
Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis.
Nature
Wu CH,Fallini C,Ticozzi N,Keagle PJ,Sapp PC,Piotrowska K,Lowe P,Koppers M,McKenna-Yasek D,Baron DM,Kost JE,Gonzalez-Perez P,Fox AD,Adams J,Taroni F,Tiloca C,Leclerc AL,Chafe SC,Mangroo D,Moore MJ,Zitzewitz JA,Xu ZS,van den Berg LH,Glass JD,Siciliano G,Cirulli ET,Goldstein DB,Salachas F,Meininger V,Rossoll W,Ratti A,Gellera C,Bosco DA,Bassell GJ,Silani V,Drory VE,Brown RH,Landers JE
Thu Aug 23 00:00:00 EDT 2012
TMEM106B risk variant is implicated in the pathologic presentation of Alzheimer disease.
Neurology
Rutherford NJ,Carrasquillo MM,Li M,Bisceglio G,Menke J,Josephs KA,Parisi JE,Petersen RC,Graff-Radford NR,Younkin SG,Dickson DW,Rademakers R
Tue Aug 14 00:00:00 EDT 2012
Targeted depletion of TDP-43 expression in the spinal cord motor neurons leads to the development of amyotrophic lateral sclerosis-like phenotypes in mice.
The Journal of biological chemistry
Wu LS,Cheng WC,Shen CK
Fri Aug 10 00:00:00 EDT 2012
Multiple system atrophy-parkinsonism with slow progression and prolonged survival: a diagnostic catch.
Movement disorders : official journal of the Movement Disorder Society
Petrovic IN,Ling H,Asi Y,Ahmed Z,Kukkle PL,Hazrati LN,Lang AE,Revesz T,Holton JL,Lees AJ
Wed Aug 01 00:00:00 EDT 2012
Distinct TDP-43 pathology in ALS patients with ataxin 2 intermediate-length polyQ expansions.
Acta neuropathologica
Hart MP,Brettschneider J,Lee VM,Trojanowski JQ,Gitler AD
Wed Aug 01 00:00:00 EDT 2012
Drug screening for ALS using patient-specific induced pluripotent stem cells.
Science translational medicine
Egawa N,Kitaoka S,Tsukita K,Naitoh M,Takahashi K,Yamamoto T,Adachi F,Kondo T,Okita K,Asaka I,Aoi T,Watanabe A,Yamada Y,Morizane A,Takahashi J,Ayaki T,Ito H,Yoshikawa K,Yamawaki S,Suzuki S,Watanabe D,Hioki H,Kaneko T,Makioka K,Okamoto K,Takuma H,Tamaoka A,Hasegawa K,Nonaka T,Hasegawa M,Kawata A,Yoshida M,Nakahata T,Takahashi R,Marchetto MC,Gage FH,Yamanaka S,Inoue H
Wed Aug 01 00:00:00 EDT 2012
Drug screening for ALS using patient-specific induced pluripotent stem cells.
Science translational medicine
Egawa N,Kitaoka S,Tsukita K,Naitoh M,Takahashi K,Yamamoto T,Adachi F,Kondo T,Okita K,Asaka I,Aoi T,Watanabe A,Yamada Y,Morizane A,Takahashi J,Ayaki T,Ito H,Yoshikawa K,Yamawaki S,Suzuki S,Watanabe D,Hioki H,Kaneko T,Makioka K,Okamoto K,Takuma H,Tamaoka A,Hasegawa K,Nonaka T,Hasegawa M,Kawata A,Yoshida M,Nakahata T,Takahashi R,Marchetto MC,Gage FH,Yamanaka S,Inoue H
Wed Aug 01 00:00:00 EDT 2012
Autoregulation of TDP-43 mRNA levels involves interplay between transcription, splicing, and alternative polyA site selection.
Genes & development
Avendaño-Vázquez SE,Dhir A,Bembich S,Buratti E,Proudfoot N,Baralle FE
Wed Aug 01 00:00:00 EDT 2012
Autoregulation of TDP-43 mRNA levels involves interplay between transcription, splicing, and alternative polyA site selection.
Genes & development
Avendaño-Vázquez SE,Dhir A,Bembich S,Buratti E,Proudfoot N,Baralle FE
Wed Aug 01 00:00:00 EDT 2012
TDP-43 pathology in a case of hereditary spastic paraplegia with a NIPA1/SPG6 mutation.
Acta neuropathologica
Martinez-Lage M,Molina-Porcel L,Falcone D,McCluskey L,Lee VM,Van Deerlin VM,Trojanowski JQ
Wed Aug 01 00:00:00 EDT 2012
Asparaginyl endopeptidase cleaves TDP-43 in brain.
Proteomics
Herskowitz JH,Gozal YM,Duong DM,Dammer EB,Gearing M,Ye K,Lah JJ,Peng J,Levey AI,Seyfried NT
Wed Aug 01 00:00:00 EDT 2012
Primary lateral sclerosis: upper-motor-predominant amyotrophic lateral sclerosis with frontotemporal lobar degeneration--immunohistochemical and biochemical analyses of TDP-43.
Neuropathology : official journal of the Japanese Society of Neuropathology
Kosaka T,Fu YJ,Shiga A,Ishidaira H,Tan CF,Tani T,Koike R,Onodera O,Nishizawa M,Kakita A,Takahashi H
Wed Aug 01 00:00:00 EDT 2012
Primary lateral sclerosis: upper-motor-predominant amyotrophic lateral sclerosis with frontotemporal lobar degeneration--immunohistochemical and biochemical analyses of TDP-43.
Neuropathology : official journal of the Japanese Society of Neuropathology
Kosaka T,Fu YJ,Shiga A,Ishidaira H,Tan CF,Tani T,Koike R,Onodera O,Nishizawa M,Kakita A,Takahashi H
Wed Aug 01 00:00:00 EDT 2012
Reduced expression of BTBD10, an Akt activator, leads to motor neuron death.
Cell death and differentiation
Nawa M,Kage-Nakadai E,Aiso S,Okamoto K,Mitani S,Matsuoka M
Wed Aug 01 00:00:00 EDT 2012
Reduced expression of BTBD10, an Akt activator, leads to motor neuron death.
Cell death and differentiation
Nawa M,Kage-Nakadai E,Aiso S,Okamoto K,Mitani S,Matsuoka M
Wed Aug 01 00:00:00 EDT 2012
Clinical features of SCA36: a novel spinocerebellar ataxia with motor neuron involvement (Asidan).
Neurology
Ikeda Y,Ohta Y,Kobayashi H,Okamoto M,Takamatsu K,Ota T,Manabe Y,Okamoto K,Koizumi A,Abe K
Tue Jul 24 00:00:00 EDT 2012
Essential tremor with ubiquitinated intranuclear inclusions and cerebellar degeneration.
Clinical neuropathology
Louis ED,Mazzoni P,Ma KJ,Moskowitz CB,Lawton A,Garber A,Vonsattel JP
Thu Jul 19 00:00:00 EDT 2012
ALS-associated ataxin 2 polyQ expansions enhance stress-induced caspase 3 activation and increase TDP-43 pathological modifications.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Hart MP,Gitler AD
Wed Jul 04 00:00:00 EDT 2012
ALS-associated ataxin 2 polyQ expansions enhance stress-induced caspase 3 activation and increase TDP-43 pathological modifications.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Hart MP,Gitler AD
Wed Jul 04 00:00:00 EDT 2012
TDP-1/TDP-43 regulates stress signaling and age-dependent proteotoxicity in Caenorhabditis elegans.
PLoS genetics
Vaccaro A,Tauffenberger A,Ash PE,Carlomagno Y,Petrucelli L,Parker JA
Sun Jul 01 00:00:00 EDT 2012
Prevalence, laterality, and comorbidity of hippocampal sclerosis in an autopsy sample.
Brain and behavior
Zarow C,Weiner MW,Ellis WG,Chui HC
Sun Jul 01 00:00:00 EDT 2012
Requirements for stress granule recruitment of fused in sarcoma (FUS) and TAR DNA-binding protein of 43 kDa (TDP-43).
The Journal of biological chemistry
Bentmann E,Neumann M,Tahirovic S,Rodde R,Dormann D,Haass C
Fri Jun 29 00:00:00 EDT 2012
Requirements for stress granule recruitment of fused in sarcoma (FUS) and TAR DNA-binding protein of 43 kDa (TDP-43).
The Journal of biological chemistry
Bentmann E,Neumann M,Tahirovic S,Rodde R,Dormann D,Haass C
Fri Jun 29 00:00:00 EDT 2012
Mutant TDP-43 and FUS cause age-dependent paralysis and neurodegeneration in C. elegans.
PloS one
Vaccaro A,Tauffenberger A,Aggad D,Rouleau G,Drapeau P,Parker JA
Fri Jun 29 00:00:00 EDT 2012
Mutant TDP-43 and FUS cause age-dependent paralysis and neurodegeneration in C. elegans.
PloS one
Vaccaro A,Tauffenberger A,Aggad D,Rouleau G,Drapeau P,Parker JA
Fri Jun 29 00:00:00 EDT 2012
Role of selected mutations in the Q/N rich region of TDP-43 in EGFP-12xQ/N-induced aggregate formation.
Brain research
Budini M,Romano V,Avendaño-Vázquez SE,Bembich S,Buratti E,Baralle FE
Tue Jun 26 00:00:00 EDT 2012
Role of selected mutations in the Q/N rich region of TDP-43 in EGFP-12xQ/N-induced aggregate formation.
Brain research
Budini M,Romano V,Avendaño-Vázquez SE,Bembich S,Buratti E,Baralle FE
Tue Jun 26 00:00:00 EDT 2012
Rodent models of TDP-43: recent advances.
Brain research
Tsao W,Jeong YH,Lin S,Ling J,Price DL,Chiang PM,Wong PC
Tue Jun 26 00:00:00 EDT 2012
HO-1 induction in motor cortex and intestinal dysfunction in TDP-43 A315T transgenic mice.
Brain research
Guo Y,Wang Q,Zhang K,An T,Shi P,Li Z,Duan W,Li C
Fri Jun 15 00:00:00 EDT 2012
Tunicamycin produces TDP-43 cytoplasmic inclusions in cultured brain organotypic slices.
Journal of the neurological sciences
Leggett C,McGehee DS,Mastrianni J,Yang W,Bai T,Brorson JR
Fri Jun 15 00:00:00 EDT 2012
The abnormal processing of TDP-43 is not an upstream event of reduced ADAR2 activity in ALS motor neurons.
Neuroscience research
Yamashita T,Hideyama T,Teramoto S,Kwak S
Fri Jun 01 00:00:00 EDT 2012
The abnormal processing of TDP-43 is not an upstream event of reduced ADAR2 activity in ALS motor neurons.
Neuroscience research
Yamashita T,Hideyama T,Teramoto S,Kwak S
Fri Jun 01 00:00:00 EDT 2012
Progressive nonfluent aphasia: a rare clinical subtype of FTLD-TDP in Japan.
Neuropathology : official journal of the Japanese Society of Neuropathology
Aoki N,Tsuchiya K,Kobayashi Z,Arai T,Togo T,Miyazaki H,Kondo H,Ishizu H,Uchikado H,Katsuse O,Hirayasu Y,Akiyama H
Fri Jun 01 00:00:00 EDT 2012
Abnormal tau deposition in neurons, but not in glial cells in the cerebral tissue surrounding arteriovenous malformation.
Neuropathology : official journal of the Japanese Society of Neuropathology
Kon T,Mori F,Tanji K,Miki Y,Tomiyama M,Baba M,Umehara Y,Kurotaki H,Wakabayashi K
Fri Jun 01 00:00:00 EDT 2012
Neuropathology in classical and variant ataxia-telangiectasia.
Neuropathology : official journal of the Japanese Society of Neuropathology
Verhagen MM,Martin JJ,van Deuren M,Ceuterick-de Groote C,Weemaes CM,Kremer BH,Taylor MA,Willemsen MA,Lammens M
Fri Jun 01 00:00:00 EDT 2012
Prevention of motor neuron degeneration by novel iron chelators in SOD1(G93A) transgenic mice of amyotrophic lateral sclerosis.
Neuro-degenerative diseases
Wang Q,Zhang X,Chen S,Zhang X,Zhang S,Youdium M,Le W
Tue May 08 00:00:00 EDT 2012
Prevention of motor neuron degeneration by novel iron chelators in SOD1(G93A) transgenic mice of amyotrophic lateral sclerosis.
Neuro-degenerative diseases
Wang Q,Zhang X,Chen S,Zhang X,Zhang S,Youdium M,Le W
Tue May 08 00:00:00 EDT 2012
TDP-43 and FUS RNA-binding proteins bind distinct sets of cytoplasmic messenger RNAs and differently regulate their post-transcriptional fate in motoneuron-like cells.
The Journal of biological chemistry
Colombrita C,Onesto E,Megiorni F,Pizzuti A,Baralle FE,Buratti E,Silani V,Ratti A
Fri May 04 00:00:00 EDT 2012
TDP-43 and FUS RNA-binding proteins bind distinct sets of cytoplasmic messenger RNAs and differently regulate their post-transcriptional fate in motoneuron-like cells.
The Journal of biological chemistry
Colombrita C,Onesto E,Megiorni F,Pizzuti A,Baralle FE,Buratti E,Silani V,Ratti A
Fri May 04 00:00:00 EDT 2012
Quantitative analysis of the detergent-insoluble brain proteome in frontotemporal lobar degeneration using SILAC internal standards.
Journal of proteome research
Seyfried NT,Gozal YM,Donovan LE,Herskowitz JH,Dammer EB,Xia Q,Ku L,Chang J,Duong DM,Rees HD,Cooper DS,Glass JD,Gearing M,Tansey MG,Lah JJ,Feng Y,Levey AI,Peng J
Fri May 04 00:00:00 EDT 2012
Quantitative analysis of the detergent-insoluble brain proteome in frontotemporal lobar degeneration using SILAC internal standards.
Journal of proteome research
Seyfried NT,Gozal YM,Donovan LE,Herskowitz JH,Dammer EB,Xia Q,Ku L,Chang J,Duong DM,Rees HD,Cooper DS,Glass JD,Gearing M,Tansey MG,Lah JJ,Feng Y,Levey AI,Peng J
Fri May 04 00:00:00 EDT 2012
Wild type TDP-43 induces neuro-inflammation and alters APP metabolism in lentiviral gene transfer models.
Experimental neurology
Herman AM,Khandelwal PJ,Rebeck GW,Moussa CE
Tue May 01 00:00:00 EDT 2012
Wild type TDP-43 induces neuro-inflammation and alters APP metabolism in lentiviral gene transfer models.
Experimental neurology
Herman AM,Khandelwal PJ,Rebeck GW,Moussa CE
Tue May 01 00:00:00 EDT 2012
First neuropathological description of a patient with Parkinson's disease and LRRK2 p.N1437H mutation.
Parkinsonism & related disorders
Puschmann A,Englund E,Ross OA,Vilariño-Güell C,Lincoln SJ,Kachergus JM,Cobb SA,Törnqvist AL,Rehncrona S,Widner H,Wszolek ZK,Farrer MJ,Nilsson C
Tue May 01 00:00:00 EDT 2012
Aquaporin-4 expression in distal myopathy with rimmed vacuoles.
BMC neurology
Hoshi A,Yamamoto T,Kikuchi S,Soeda T,Shimizu K,Ugawa Y
Fri Apr 27 00:00:00 EDT 2012
Comparison of phosphorylated TDP-43-positive inclusions in oculomotor neurons in patients with non-ALS and ALS disorders.
Journal of the neurological sciences
Mizuno Y,Fujita Y,Takatama M,Okamoto K
Sun Apr 15 00:00:00 EDT 2012
Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability.
Proceedings of the National Academy of Sciences of the United States of America
Bilican B,Serio A,Barmada SJ,Nishimura AL,Sullivan GJ,Carrasco M,Phatnani HP,Puddifoot CA,Story D,Fletcher J,Park IH,Friedman BA,Daley GQ,Wyllie DJ,Hardingham GE,Wilmut I,Finkbeiner S,Maniatis T,Shaw CE,Chandran S
Tue Apr 10 00:00:00 EDT 2012
Phosphorylated tau/amyloid beta 1-42 ratio in ventricular cerebrospinal fluid reflects outcome in idiopathic normal pressure hydrocephalus.
Fluids and barriers of the CNS
Patel S,Lee EB,Xie SX,Law A,Jackson EM,Arnold SE,Clark CM,Shaw LM,Grady MS,Trojanowski JQ,Hamilton RH
Fri Mar 23 00:00:00 EDT 2012
Caenorhabditis elegans RNA-processing protein TDP-1 regulates protein homeostasis and life span.
The Journal of biological chemistry
Zhang T,Hwang HY,Hao H,Talbot C,Wang J
Fri Mar 09 00:00:00 EST 2012
Redox signalling directly regulates TDP-43 via cysteine oxidation and disulphide cross-linking.
The EMBO journal
Cohen TJ,Hwang AW,Unger T,Trojanowski JQ,Lee VM
Wed Mar 07 00:00:00 EST 2012
Redox signalling directly regulates TDP-43 via cysteine oxidation and disulphide cross-linking.
The EMBO journal
Cohen TJ,Hwang AW,Unger T,Trojanowski JQ,Lee VM
Wed Mar 07 00:00:00 EST 2012
Cellular model of TAR DNA-binding protein 43 (TDP-43) aggregation based on its C-terminal Gln/Asn-rich region.
The Journal of biological chemistry
Budini M,Buratti E,Stuani C,Guarnaccia C,Romano V,De Conti L,Baralle FE
Fri Mar 02 00:00:00 EST 2012
Non-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-43.
Brain : a journal of neurology
Uchida A,Sasaguri H,Kimura N,Tajiri M,Ohkubo T,Ono F,Sakaue F,Kanai K,Hirai T,Sano T,Shibuya K,Kobayashi M,Yamamoto M,Yokota S,Kubodera T,Tomori M,Sakaki K,Enomoto M,Hirai Y,Kumagai J,Yasutomi Y,Mochizuki H,Kuwabara S,Uchihara T,Mizusawa H,Yokota T
Thu Mar 01 00:00:00 EST 2012
Non-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-43.
Brain : a journal of neurology
Uchida A,Sasaguri H,Kimura N,Tajiri M,Ohkubo T,Ono F,Sakaue F,Kanai K,Hirai T,Sano T,Shibuya K,Kobayashi M,Yamamoto M,Yokota S,Kubodera T,Tomori M,Sakaki K,Enomoto M,Hirai Y,Kumagai J,Yasutomi Y,Mochizuki H,Kuwabara S,Uchihara T,Mizusawa H,Yokota T
Thu Mar 01 00:00:00 EST 2012
Endogenous TDP-43 localized to stress granules can subsequently form protein aggregates.
Neurochemistry international
Parker SJ,Meyerowitz J,James JL,Liddell JR,Crouch PJ,Kanninen KM,White AR
Thu Mar 01 00:00:00 EST 2012
Clinical and pathological features of amyotrophic lateral sclerosis caused by mutation in the C9ORF72 gene on chromosome 9p.
Acta neuropathologica
Stewart H,Rutherford NJ,Briemberg H,Krieger C,Cashman N,Fabros M,Baker M,Fok A,DeJesus-Hernandez M,Eisen A,Rademakers R,Mackenzie IR
Thu Mar 01 00:00:00 EST 2012
Exome sequencing reveals DNAJB6 mutations in dominantly-inherited myopathy.
Annals of neurology
Harms MB,Sommerville RB,Allred P,Bell S,Ma D,Cooper P,Lopate G,Pestronk A,Weihl CC,Baloh RH
Thu Mar 01 00:00:00 EST 2012
Progressive neuronal inclusion formation and axonal degeneration in CHMP2B mutant transgenic mice.
Brain : a journal of neurology
Ghazi-Noori S,Froud KE,Mizielinska S,Powell C,Smidak M,Fernandez de Marco M,O'Malley C,Farmer M,Parkinson N,Fisher EM,Asante EA,Brandner S,Collinge J,Isaacs AM
Thu Mar 01 00:00:00 EST 2012
TDP-43 regulates global translational yield by splicing of exon junction complex component SKAR.
Nucleic acids research
Fiesel FC,Weber SS,Supper J,Zell A,Kahle PJ
Thu Mar 01 00:00:00 EST 2012
Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations.
Brain : a journal of neurology
Snowden JS,Rollinson S,Thompson JC,Harris JM,Stopford CL,Richardson AM,Jones M,Gerhard A,Davidson YS,Robinson A,Gibbons L,Hu Q,DuPlessis D,Neary D,Mann DM,Pickering-Brown SM
Thu Mar 01 00:00:00 EST 2012
Oxidative stress induced by glutathione depletion reproduces pathological modifications of TDP-43 linked to TDP-43 proteinopathies.
Neurobiology of disease
Iguchi Y,Katsuno M,Takagi S,Ishigaki S,Niwa J,Hasegawa M,Tanaka F,Sobue G
Thu Mar 01 00:00:00 EST 2012
Oxidative stress induced by glutathione depletion reproduces pathological modifications of TDP-43 linked to TDP-43 proteinopathies.
Neurobiology of disease
Iguchi Y,Katsuno M,Takagi S,Ishigaki S,Niwa J,Hasegawa M,Tanaka F,Sobue G
Thu Mar 01 00:00:00 EST 2012
Clinical and pathological features of familial frontotemporal dementia caused by C9ORF72 mutation on chromosome 9p.
Brain : a journal of neurology
Hsiung GY,DeJesus-Hernandez M,Feldman HH,Sengdy P,Bouchard-Kerr P,Dwosh E,Butler R,Leung B,Fok A,Rutherford NJ,Baker M,Rademakers R,Mackenzie IR
Thu Mar 01 00:00:00 EST 2012
TDP-43 promotes microRNA biogenesis as a component of the Drosha and Dicer complexes.
Proceedings of the National Academy of Sciences of the United States of America
Kawahara Y,Mieda-Sato A
Tue Feb 28 00:00:00 EST 2012
Unusual clinical presentation and neuropathology in two subjects with fused-in sarcoma (FUS) positive inclusions.
Neuropathology : official journal of the Japanese Society of Neuropathology
Hartikainen PH,Pikkarainen M,Hänninen T,Soininen H,Alafuzoff I
Wed Feb 01 00:00:00 EST 2012
A quantitative study of the neuropathology of 32 sporadic and familial cases of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP).
Neuropathology and applied neurobiology
Armstrong RA,Carter D,Cairns NJ
Wed Feb 01 00:00:00 EST 2012
Alteration in cell cycle-related proteins in lymphoblasts from carriers of the c.709-1G>A PGRN mutation associated with FTLD-TDP dementia.
Neurobiology of aging
Alquezar C,Esteras N,Bartolomé F,Merino JJ,Alzualde A,López de Munain A,Martín-Requero Á
Wed Feb 01 00:00:00 EST 2012
Alteration in cell cycle-related proteins in lymphoblasts from carriers of the c.709-1G>A PGRN mutation associated with FTLD-TDP dementia.
Neurobiology of aging
Alquezar C,Esteras N,Bartolomé F,Merino JJ,Alzualde A,López de Munain A,Martín-Requero Á
Wed Feb 01 00:00:00 EST 2012
Epitope mapping of antibodies against TDP-43 and detection of protease-resistant fragments of pathological TDP-43 in amyotrophic lateral sclerosis and frontotemporal lobar degeneration.
Biochemical and biophysical research communications
Tsuji H,Nonaka T,Yamashita M,Masuda-Suzukake M,Kametani F,Akiyama H,Mann DM,Tamaoka A,Hasegawa M
Fri Jan 06 00:00:00 EST 2012
National Institute on Aging-Alzheimer's Association guidelines for the neuropathologic assessment of Alzheimer's disease: a practical approach.
Acta neuropathologica
Montine TJ,Phelps CH,Beach TG,Bigio EH,Cairns NJ,Dickson DW,Duyckaerts C,Frosch MP,Masliah E,Mirra SS,Nelson PT,Schneider JA,Thal DR,Trojanowski JQ,Vinters HV,Hyman BT
Sun Jan 01 00:00:00 EST 2012
Cognitive decline typical of frontotemporal lobar degeneration in transgenic mice expressing the 25-kDa C-terminal fragment of TDP-43.
The American journal of pathology
Caccamo A,Majumder S,Oddo S
Sun Jan 01 00:00:00 EST 2012
A role for SMN exon 7 splicing in the selective vulnerability of motor neurons in spinal muscular atrophy.
Molecular and cellular biology
Ruggiu M,McGovern VL,Lotti F,Saieva L,Li DK,Kariya S,Monani UR,Burghes AH,Pellizzoni L
Sun Jan 01 00:00:00 EST 2012
Transglutaminase 1 and its regulator tazarotene-induced gene 3 localize to neuronal tau inclusions in tauopathies.
The Journal of pathology
Wilhelmus MM,de Jager M,Rozemuller AJ,Brevé J,Bol JG,Eckert RL,Drukarch B
Sun Jan 01 00:00:00 EST 2012
Supranuclear ophthalmoparesis and vacuolar degeneration of the cerebral white matter in amyotrophic lateral sclerosis: a clinicopathological study.
Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
Takeda T,Uchihara T,Mochizuki Y,Ishihara A,Nakamura A,Sasaki S,Uchiyama S,Iwata M,Mizutani T
Sun Jan 01 00:00:00 EST 2012
Absence of SOD1 leads to oxidative stress in peripheral nerve and causes a progressive distal motor axonopathy.
Experimental neurology
Fischer LR,Li Y,Asress SA,Jones DP,Glass JD
Sun Jan 01 00:00:00 EST 2012
Right temporal variant frontotemporal dementia with motor neuron disease.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
Coon EA,Whitwell JL,Parisi JE,Dickson DW,Josephs KA
Sun Jan 01 00:00:00 EST 2012
A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study.
The Lancet. Neurology
Gijselinck I,Van Langenhove T,van der Zee J,Sleegers K,Philtjens S,Kleinberger G,Janssens J,Bettens K,Van Cauwenberghe C,Pereson S,Engelborghs S,Sieben A,De Jonghe P,Vandenberghe R,Santens P,De Bleecker J,Maes G,Bäumer V,Dillen L,Joris G,Cuijt I,Corsmit E,Elinck E,Van Dongen J,Vermeulen S,Van den Broeck M,Vaerenberg C,Mattheijssens M,Peeters K,Robberecht W,Cras P,Martin JJ,De Deyn PP,Cruts M,Van Broeckhoven C
Sun Jan 01 00:00:00 EST 2012
Diacetylbis(N(4)-methylthiosemicarbazonato) copper(II) (CuII(atsm)) protects against peroxynitrite-induced nitrosative damage and prolongs survival in amyotrophic lateral sclerosis mouse model.
The Journal of biological chemistry
Soon CPW,Donnelly PS,Turner BJ,Hung LW,Crouch PJ,Sherratt NA,Tan JL,Lim NK,Lam L,Bica L,Lim S,Hickey JL,Morizzi J,Powell A,Finkelstein DI,Culvenor JG,Masters CL,Duce J,White AR,Barnham KJ,Li QX
Fri Dec 23 00:00:00 EST 2011
Cytoplasmic accumulation and aggregation of TDP-43 upon proteasome inhibition in cultured neurons.
PloS one
van Eersel J,Ke YD,Gladbach A,Bi M,Götz J,Kril JJ,Ittner LM
Tue Dec 06 00:00:00 EST 2011
Cytoplasmic accumulation and aggregation of TDP-43 upon proteasome inhibition in cultured neurons.
PloS one
van Eersel J,Ke YD,Gladbach A,Bi M,Götz J,Kril JJ,Ittner LM
Tue Dec 06 00:00:00 EST 2011
TDP-43 pathological changes in early onset familial and sporadic Alzheimer's disease, late onset Alzheimer's disease and Down's syndrome: association with age, hippocampal sclerosis and clinical phenotype.
Acta neuropathologica
Davidson YS,Raby S,Foulds PG,Robinson A,Thompson JC,Sikkink S,Yusuf I,Amin H,DuPlessis D,Troakes C,Al-Sarraj S,Sloan C,Esiri MM,Prasher VP,Allsop D,Neary D,Pickering-Brown SM,Snowden JS,Mann DM
Thu Dec 01 00:00:00 EST 2011
Acute and chronically increased immunoreactivity to phosphorylation-independent but not pathological TDP-43 after a single traumatic brain injury in humans.
Acta neuropathologica
Johnson VE,Stewart W,Trojanowski JQ,Smith DH
Thu Dec 01 00:00:00 EST 2011
The ALS/PDC syndrome of Guam: potential biomarkers for an enigmatic disorder.
Progress in neurobiology
McGeer PL,Steele JC
Thu Dec 01 00:00:00 EST 2011
Molecular properties of TAR DNA binding protein-43 fragments are dependent upon its cleavage site.
Biochimica et biophysica acta
Furukawa Y,Kaneko K,Nukina N
Thu Dec 01 00:00:00 EST 2011
Abnormal TDP-43 and FUS proteins in muscles of sporadic IBM: similarities in a TARDBP-linked ALS patient.
Journal of neurology, neurosurgery, and psychiatry
Hernandez Lain A,Millecamps S,Dubourg O,Salachas F,Bruneteau G,Lacomblez L,LeGuern E,Seilhean D,Duyckaerts C,Meininger V,Mallet J,Pradat PF
Thu Dec 01 00:00:00 EST 2011
An autopsy case of SOD1-related ALS with TDP-43 positive inclusions.
Neurology
Okamoto Y,Ihara M,Urushitani M,Yamashita H,Kondo T,Tanigaki A,Oono M,Kawamata J,Ikemoto A,Kawamoto Y,Takahashi R,Ito H
Tue Nov 29 00:00:00 EST 2011
Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor κB-mediated pathogenic pathways.
The Journal of experimental medicine
Swarup V,Phaneuf D,Dupré N,Petri S,Strong M,Kriz J,Julien JP
Mon Nov 21 00:00:00 EST 2011
Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor κB-mediated pathogenic pathways.
The Journal of experimental medicine
Swarup V,Phaneuf D,Dupré N,Petri S,Strong M,Kriz J,Julien JP
Mon Nov 21 00:00:00 EST 2011
TDP-43 variants of frontotemporal lobar degeneration.
Journal of molecular neuroscience : MN
Bigio EH
Tue Nov 01 00:00:00 EDT 2011
Long-term observational study of sporadic inclusion body myositis.
Brain : a journal of neurology
Benveniste O,Guiguet M,Freebody J,Dubourg O,Squier W,Maisonobe T,Stojkovic T,Leite MI,Allenbach Y,Herson S,Brady S,Eymard B,Hilton-Jones D
Tue Nov 01 00:00:00 EDT 2011
Astrocytes carrying the superoxide dismutase 1 (SOD1G93A) mutation induce wild-type motor neuron degeneration in vivo.
Proceedings of the National Academy of Sciences of the United States of America
Papadeas ST,Kraig SE,O'Banion C,Lepore AC,Maragakis NJ
Tue Oct 25 00:00:00 EDT 2011
Novel progranulin mutation detected in 2 patients with FTLD.
Alzheimer disease and associated disorders
Skoglund L,Matsui T,Freeman SH,Wallin A,Blom ES,Frosch MP,Growdon JH,Hyman BT,Lannfelt L,Ingelsson M,Glaser A
Wed Oct 19 00:00:00 EDT 2011
An autopsy case of an aged patient with spinocerebellar ataxia type 2.
Neuropathology : official journal of the Japanese Society of Neuropathology
Ishida C,Komai K,Yonezawa K,Sakajiri K,Nitta E,Kawashima A,Yamada M
Sat Oct 01 00:00:00 EDT 2011
Immunohistochemical study of microscopic globular bodies of normal human brain.
Biomedical research (Tokyo, Japan)
Odagiri S,Mori F,Tanji K,Kuroda N,Wakabayashi K
Sat Oct 01 00:00:00 EDT 2011
Diagnostic value of markers of muscle degeneration in sporadic inclusion body myositis.
Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology
Dubourg O,Wanschitz J,Maisonobe T,Béhin A,Allenbach Y,Herson S,Benveniste O
Sat Oct 01 00:00:00 EDT 2011
Proportional neuronal loss between the primary motor and sensory cortex in amyotrophic lateral sclerosis.
Neuroscience letters
Mochizuki Y,Mizutani T,Shimizu T,Kawata A
Mon Sep 26 00:00:00 EDT 2011
Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments.
Brain : a journal of neurology
Swarup V,Phaneuf D,Bareil C,Robertson J,Rouleau GA,Kriz J,Julien JP
Thu Sep 01 00:00:00 EDT 2011
Spinocerebellar ataxia type 2 (SCA2) is associated with TDP-43 pathology.
Acta neuropathologica
Toyoshima Y,Tanaka H,Shimohata M,Kimura K,Morita T,Kakita A,Takahashi H
Thu Sep 01 00:00:00 EDT 2011
Temporoparietal atrophy: a marker of AD pathology independent of clinical diagnosis.
Neurobiology of aging
Whitwell JL,Jack CR,Przybelski SA,Parisi JE,Senjem ML,Boeve BF,Knopman DS,Petersen RC,Dickson DW,Josephs KA
Thu Sep 01 00:00:00 EDT 2011
A comprehensive assessment of the SOD1G93A low-copy transgenic mouse, which models human amyotrophic lateral sclerosis.
Disease models & mechanisms
Acevedo-Arozena A,Kalmar B,Essa S,Ricketts T,Joyce P,Kent R,Rowe C,Parker A,Gray A,Hafezparast M,Thorpe JR,Greensmith L,Fisher EM
Thu Sep 01 00:00:00 EDT 2011
The clinical diagnosis of early-onset dementias: diagnostic accuracy and clinicopathological relationships.
Brain : a journal of neurology
Snowden JS,Thompson JC,Stopford CL,Richardson AM,Gerhard A,Neary D,Mann DM
Thu Sep 01 00:00:00 EDT 2011
TDP-43 knockdown impairs neurite outgrowth dependent on its target histone deacetylase 6.
Molecular neurodegeneration
Fiesel FC,Schurr C,Weber SS,Kahle PJ
Tue Aug 30 00:00:00 EDT 2011
Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia.
Nature
Deng HX,Chen W,Hong ST,Boycott KM,Gorrie GH,Siddique N,Yang Y,Fecto F,Shi Y,Zhai H,Jiang H,Hirano M,Rampersaud E,Jansen GH,Donkervoort S,Bigio EH,Brooks BR,Ajroud K,Sufit RL,Haines JL,Mugnaini E,Pericak-Vance MA,Siddique T
Sun Aug 21 00:00:00 EDT 2011
C-Jun N-terminal kinase controls TDP-43 accumulation in stress granules induced by oxidative stress.
Molecular neurodegeneration
Meyerowitz J,Parker SJ,Vella LJ,Ng DCh,Price KA,Liddell JR,Caragounis A,Li QX,Masters CL,Nonaka T,Hasegawa M,Bogoyevitch MA,Kanninen KM,Crouch PJ,White AR
Mon Aug 08 00:00:00 EDT 2011
C-Jun N-terminal kinase controls TDP-43 accumulation in stress granules induced by oxidative stress.
Molecular neurodegeneration
Meyerowitz J,Parker SJ,Vella LJ,Ng DCh,Price KA,Liddell JR,Caragounis A,Li QX,Masters CL,Nonaka T,Hasegawa M,Bogoyevitch MA,Kanninen KM,Crouch PJ,White AR
Mon Aug 08 00:00:00 EDT 2011
FUS and TARDBP but not SOD1 interact in genetic models of amyotrophic lateral sclerosis.
PLoS genetics
Kabashi E,Bercier V,Lissouba A,Liao M,Brustein E,Rouleau GA,Drapeau P
Mon Aug 01 00:00:00 EDT 2011
Clinicopathologic study on an ALS family with a heterozygous E478G optineurin mutation.
Acta neuropathologica
Ito H,Nakamura M,Komure O,Ayaki T,Wate R,Maruyama H,Nakamura Y,Fujita K,Kaneko S,Okamoto Y,Ihara M,Konishi T,Ogasawara K,Hirano A,Kusaka H,Kaji R,Takahashi R,Kawakami H
Mon Aug 01 00:00:00 EDT 2011
A peculiar constellation of tau pathology defines a subset of dementia in the elderly.
Acta neuropathologica
Kovacs GG,Molnár K,László L,Ströbel T,Botond G,Hönigschnabl S,Reiner-Concin A,Palkovits M,Fischer P,Budka H
Mon Aug 01 00:00:00 EDT 2011
Enhancement of native and phosphorylated TDP-43 immunoreactivity by proteinase K treatment following autoclave heating.
Neuropathology : official journal of the Japanese Society of Neuropathology
Mori F,Tanji K,Kakita A,Takahashi H,Wakabayashi K
Mon Aug 01 00:00:00 EDT 2011
Spatial patterns of TDP-43 neuronal cytoplasmic inclusions (NCI) in fifteen cases of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP).
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
Armstrong RA,Cairns NJ
Mon Aug 01 00:00:00 EDT 2011
Differential involvement of optineurin in amyotrophic lateral sclerosis with or without SOD1 mutations.
Archives of neurology
Deng HX,Bigio EH,Zhai H,Fecto F,Ajroud K,Shi Y,Yan J,Mishra M,Ajroud-Driss S,Heller S,Sufit R,Siddique N,Mugnaini E,Siddique T
Mon Aug 01 00:00:00 EDT 2011
Differential involvement of optineurin in amyotrophic lateral sclerosis with or without SOD1 mutations.
Archives of neurology
Deng HX,Bigio EH,Zhai H,Fecto F,Ajroud K,Shi Y,Yan J,Mishra M,Ajroud-Driss S,Heller S,Sufit R,Siddique N,Mugnaini E,Siddique T
Mon Aug 01 00:00:00 EDT 2011
Proteomic analysis of hippocampal dentate granule cells in frontotemporal lobar degeneration: application of laser capture technology.
Frontiers in neurology
Gozal YM,Dammer EB,Duong DM,Cheng D,Gearing M,Rees HD,Peng J,Lah JJ,Levey AI
Thu Jul 14 00:00:00 EDT 2011
The most common type of FTLD-FUS (aFTLD-U) is associated with a distinct clinical form of frontotemporal dementia but is not related to mutations in the FUS gene.
Acta neuropathologica
Snowden JS,Hu Q,Rollinson S,Halliwell N,Robinson A,Davidson YS,Momeni P,Baborie A,Griffiths TD,Jaros E,Perry RH,Richardson A,Pickering-Brown SM,Neary D,Mann DM
Fri Jul 01 00:00:00 EDT 2011
RNA targets of TDP-43 identified by UV-CLIP are deregulated in ALS.
Molecular and cellular neurosciences
Xiao S,Sanelli T,Dib S,Sheps D,Findlater J,Bilbao J,Keith J,Zinman L,Rogaeva E,Robertson J
Fri Jul 01 00:00:00 EDT 2011
A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43.
Human molecular genetics
Lanson NA,Maltare A,King H,Smith R,Kim JH,Taylor JP,Lloyd TE,Pandey UB
Fri Jul 01 00:00:00 EDT 2011
An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxicity.
Nature structural & molecular biology
Guo W,Chen Y,Zhou X,Kar A,Ray P,Chen X,Rao EJ,Yang M,Ye H,Zhu L,Liu J,Xu M,Yang Y,Wang C,Zhang D,Bigio EH,Mesulam M,Shen Y,Xu Q,Fushimi K,Wu JY
Sun Jun 12 00:00:00 EDT 2011
An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxicity.
Nature structural & molecular biology
Guo W,Chen Y,Zhou X,Kar A,Ray P,Chen X,Rao EJ,Yang M,Ye H,Zhu L,Liu J,Xu M,Yang Y,Wang C,Zhang D,Bigio EH,Mesulam M,Shen Y,Xu Q,Fushimi K,Wu JY
Sun Jun 12 00:00:00 EDT 2011
TDP-43 pathology occurs infrequently in multiple system atrophy.
Neuropathology and applied neurobiology
Geser F,Malunda JA,Hurtig HI,Duda JE,Wenning GK,Gilman S,Low PA,Lee VM,Trojanowski JQ
Wed Jun 01 00:00:00 EDT 2011
Semantic dementia with lower motor neuron disease showing FTLD-TDP type 3 pathology (sensu Mackenzie).
Neuropathology : official journal of the Japanese Society of Neuropathology
Östberg P,Bogdanović N
Wed Jun 01 00:00:00 EDT 2011
Catastrophic hyperkalaemia following administration of suxamethonium chloride to a patient with undiagnosed amyotrophic lateral sclerosis.
Clinical medicine (London, England)
Turner MR,Lawrence H,Arnold I,Ansorge O,Talbot K
Wed Jun 01 00:00:00 EDT 2011
Ubiquitinated, p62 immunopositive cerebellar cortical neuronal inclusions are evident across the spectrum of TDP-43 proteinopathies but are only rarely additionally immunopositive for phosphorylation-dependent TDP-43.
Neuropathology : official journal of the Japanese Society of Neuropathology
King A,Maekawa S,Bodi I,Troakes C,Al-Sarraj S
Wed Jun 01 00:00:00 EDT 2011
Intra-familial clinical heterogeneity due to FTLD-U with TDP-43 proteinopathy caused by a novel deletion in progranulin gene (PGRN).
Journal of Alzheimer's disease : JAD
Gabryelewicz T,Masellis M,Berdynski M,Bilbao JM,Rogaeva E,St George-Hyslop P,Barczak A,Czyzewski K,Barcikowska M,Wszolek Z,Black SE,Zekanowski C
Wed May 25 00:00:00 EDT 2011
TDP-43 neurotoxicity and protein aggregation modulated by heat shock factor and insulin/IGF-1 signaling.
Human molecular genetics
Zhang T,Mullane PC,Periz G,Wang J
Sun May 15 00:00:00 EDT 2011
FTLD-TDP with motor neuron disease, visuospatial impairment and a progressive supranuclear palsy-like syndrome: broadening the clinical phenotype of TDP-43 proteinopathies. A report of three cases.
BMC neurology
Rusina R,Kovacs GG,Fiala J,Hort J,Ridzoň P,Holmerová I,Ströbel T,Matěj R
Tue May 10 00:00:00 EDT 2011
A family with Parkinsonism, essential tremor, restless legs syndrome, and depression.
Neurology
Puschmann A,Pfeiffer RF,Stoessl AJ,Kuriakose R,Lash JL,Searcy JA,Strongosky AJ,Vilariño-Güell C,Farrer MJ,Ross OA,Dickson DW,Wszolek ZK
Tue May 10 00:00:00 EDT 2011
Fronto-temporal lobar degeneration: neuropathology in 60 cases.
Journal of neural transmission (Vienna, Austria : 1996)
Seilhean D,Le Ber I,Sarazin M,Lacomblez L,Millecamps S,Salachas F,Pradat PF,Le Forestier N,LeGuern E,Dubois B,Meininger V,Brice A,Hauw JJ,Duyckaerts C
Sun May 01 00:00:00 EDT 2011
Granular expression of prolyl-peptidyl isomerase PIN1 is a constant and specific feature of Alzheimer's disease pathology and is independent of tau, Aβ and TDP-43 pathology.
Acta neuropathologica
Dakson A,Yokota O,Esiri M,Bigio EH,Horan M,Pendleton N,Richardson A,Neary D,Snowden JS,Robinson A,Davidson YS,Mann DM
Sun May 01 00:00:00 EDT 2011
Hippocampal sclerosis in advanced age: clinical and pathological features.
Brain : a journal of neurology
Nelson PT,Schmitt FA,Lin Y,Abner EL,Jicha GA,Patel E,Thomason PC,Neltner JH,Smith CD,Santacruz KS,Sonnen JA,Poon LW,Gearing M,Green RC,Woodard JL,Van Eldik LJ,Kryscio RJ
Sun May 01 00:00:00 EDT 2011
β-amyloid triggers ALS-associated TDP-43 pathology in AD models.
Brain research
Herman AM,Khandelwal PJ,Stanczyk BB,Rebeck GW,Moussa CE
Fri Apr 22 00:00:00 EDT 2011
β-amyloid triggers ALS-associated TDP-43 pathology in AD models.
Brain research
Herman AM,Khandelwal PJ,Stanczyk BB,Rebeck GW,Moussa CE
Fri Apr 22 00:00:00 EDT 2011
TDP-43-induced death is associated with altered regulation of BIM and Bcl-xL and attenuated by caspase-mediated TDP-43 cleavage.
The Journal of biological chemistry
Suzuki H,Lee K,Matsuoka M
Fri Apr 15 00:00:00 EDT 2011
Novel histopathologic findings in molecularly-confirmed pantothenate kinase-associated neurodegeneration.
Brain : a journal of neurology
Kruer MC,Hiken M,Gregory A,Malandrini A,Clark D,Hogarth P,Grafe M,Hayflick SJ,Woltjer RL
Fri Apr 01 00:00:00 EDT 2011
Characterizing the RNA targets and position-dependent splicing regulation by TDP-43.
Nature neuroscience
Tollervey JR,Curk T,Rogelj B,Briese M,Cereda M,Kayikci M,König J,Hortobágyi T,Nishimura AL,Zupunski V,Patani R,Chandran S,Rot G,Zupan B,Shaw CE,Ule J
Fri Apr 01 00:00:00 EDT 2011
Motor neuron disease clinically limited to the lower motor neuron is a diffuse TDP-43 proteinopathy.
Acta neuropathologica
Geser F,Stein B,Partain M,Elman LB,McCluskey LF,Xie SX,Van Deerlin VM,Kwong LK,Lee VM,Trojanowski JQ
Fri Apr 01 00:00:00 EDT 2011
Familial frontotemporal dementia with amyotrophic lateral sclerosis and a shared haplotype on chromosome 9p.
Journal of neurology
Pearson JP,Williams NM,Majounie E,Waite A,Stott J,Newsway V,Murray A,Hernandez D,Guerreiro R,Singleton AB,Neal J,Morris HR
Fri Apr 01 00:00:00 EDT 2011
TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1.
Human molecular genetics
McDonald KK,Aulas A,Destroismaisons L,Pickles S,Beleac E,Camu W,Rouleau GA,Vande Velde C
Fri Apr 01 00:00:00 EDT 2011
TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1.
Human molecular genetics
McDonald KK,Aulas A,Destroismaisons L,Pickles S,Beleac E,Camu W,Rouleau GA,Vande Velde C
Fri Apr 01 00:00:00 EDT 2011
Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43.
Nature neuroscience
Polymenidou M,Lagier-Tourenne C,Hutt KR,Huelga SC,Moran J,Liang TY,Ling SC,Sun E,Wancewicz E,Mazur C,Kordasiewicz H,Sedaghat Y,Donohue JP,Shiue L,Bennett CF,Yeo GW,Cleveland DW
Fri Apr 01 00:00:00 EDT 2011
Familial prion disease with Alzheimer disease-like tau pathology and clinical phenotype.
Annals of neurology
Jayadev S,Nochlin D,Poorkaj P,Steinbart EJ,Mastrianni JA,Montine TJ,Ghetti B,Schellenberg GD,Bird TD,Leverenz JB
Fri Apr 01 00:00:00 EDT 2011
TDP-43 pathology may occur in the BRI2 gene-related dementias.
Acta neuropathologica
Lashley T,Holton JL,Revesz T
Fri Apr 01 00:00:00 EDT 2011
In sporadic inclusion body myositis muscle fibres TDP-43-positive inclusions are less frequent and robust than p62 inclusions, and are not associated with paired helical filaments.
Neuropathology and applied neurobiology
D'Agostino C,Nogalska A,Engel WK,Askanas V
Fri Apr 01 00:00:00 EDT 2011
TDP-43 potentiates alpha-synuclein toxicity to dopaminergic neurons in transgenic mice.
International journal of biological sciences
Tian T,Huang C,Tong J,Yang M,Zhou H,Xia XG
Fri Mar 04 00:00:00 EST 2011
TDP-43 is directed to stress granules by sorbitol, a novel physiological osmotic and oxidative stressor.
Molecular and cellular biology
Dewey CM,Cenik B,Sephton CF,Dries DR,Mayer P,Good SK,Johnson BA,Herz J,Yu G
Tue Mar 01 00:00:00 EST 2011
TDP-43 is directed to stress granules by sorbitol, a novel physiological osmotic and oxidative stressor.
Molecular and cellular biology
Dewey CM,Cenik B,Sephton CF,Dries DR,Mayer P,Good SK,Johnson BA,Herz J,Yu G
Tue Mar 01 00:00:00 EST 2011
Elevated CSF TDP-43 levels in amyotrophic lateral sclerosis: specificity, sensitivity, and a possible prognostic value.
Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
Noto Y,Shibuya K,Sato Y,Kanai K,Misawa S,Sawai S,Mori M,Uchiyama T,Isose S,Nasu S,Sekiguchi Y,Fujimaki Y,Kasai T,Tokuda T,Nakagawa M,Kuwabara S
Tue Mar 01 00:00:00 EST 2011
New neuropathological findings in Unverricht-Lundborg disease: neuronal intranuclear and cytoplasmic inclusions.
Acta neuropathologica
Cohen NR,Hammans SR,Macpherson J,Nicoll JA
Tue Mar 01 00:00:00 EST 2011
A novel double mutation in FUS gene causing sporadic ALS.
Neurobiology of aging
Robertson J,Bilbao J,Zinman L,Hazrati LN,Tokuhiro S,Sato C,Moreno D,Strome R,Mackenzie IR,Rogaeva E
Tue Mar 01 00:00:00 EST 2011
Pathological correlates of frontotemporal lobar degeneration in the elderly.
Acta neuropathologica
Baborie A,Griffiths TD,Jaros E,McKeith IG,Burn DJ,Richardson A,Ferrari R,Moreno J,Momeni P,Duplessis D,Pal P,Rollinson S,Pickering-Brown S,Thompson JC,Neary D,Snowden JS,Perry R,Mann DM
Tue Mar 01 00:00:00 EST 2011
Inorganic mercury within motor neurons does not cause the TDP-43 changes seen in sporadic ALS.
Toxicology letters
Pamphlett R,Kum Jew S
Fri Feb 25 00:00:00 EST 2011
Clinicopathological study of diffuse neurofibrillary tangles with calcification. With special reference to TDP-43 proteinopathy and alpha-synucleinopathy.
Journal of the neurological sciences
Habuchi C,Iritani S,Sekiguchi H,Torii Y,Ishihara R,Arai T,Hasegawa M,Tsuchiya K,Akiyama H,Shibayama H,Ozaki N
Tue Feb 15 00:00:00 EST 2011
Sensory and motor neuronopathy in a patient with the A382P TDP-43 mutation.
Orphanet journal of rare diseases
Camdessanché JP,Belzil VV,Jousserand G,Rouleau GA,Créac'h C,Convers P,Antoine JC
Sat Feb 05 00:00:00 EST 2011
Clinical, neuroimaging and neuropathological features of a new chromosome 9p-linked FTD-ALS family.
Journal of neurology, neurosurgery, and psychiatry
Boxer AL,Mackenzie IR,Boeve BF,Baker M,Seeley WW,Crook R,Feldman H,Hsiung GY,Rutherford N,Laluz V,Whitwell J,Foti D,McDade E,Molano J,Karydas A,Wojtas A,Goldman J,Mirsky J,Sengdy P,Dearmond S,Miller BL,Rademakers R
Tue Feb 01 00:00:00 EST 2011
Both cytoplasmic and nuclear accumulations of the protein are neurotoxic in Drosophila models of TDP-43 proteinopathies.
Neurobiology of disease
Miguel L,Frébourg T,Campion D,Lecourtois M
Tue Feb 01 00:00:00 EST 2011
Both cytoplasmic and nuclear accumulations of the protein are neurotoxic in Drosophila models of TDP-43 proteinopathies.
Neurobiology of disease
Miguel L,Frébourg T,Campion D,Lecourtois M
Tue Feb 01 00:00:00 EST 2011
A morphometric study of the spatial patterns of TDP-43 immunoreactive neuronal inclusions in frontotemporal lobar degeneration (FTLD) with progranulin (GRN) mutation.
Histology and histopathology
Armstrong RA,Cairns NJ
Tue Feb 01 00:00:00 EST 2011
Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice.
The Journal of clinical investigation
Igaz LM,Kwong LK,Lee EB,Chen-Plotkin A,Swanson E,Unger T,Malunda J,Xu Y,Winton MJ,Trojanowski JQ,Lee VM
Tue Feb 01 00:00:00 EST 2011
Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice.
The Journal of clinical investigation
Igaz LM,Kwong LK,Lee EB,Chen-Plotkin A,Swanson E,Unger T,Malunda J,Xu Y,Winton MJ,Trojanowski JQ,Lee VM
Tue Feb 01 00:00:00 EST 2011
TDP43-positive intraneuronal inclusions in a patient with motor neuron disease and Parkinson's disease.
Neuro-degenerative diseases
Chanson JB,Echaniz-Laguna A,Vogel T,Mohr M,Benoilid A,Kaltenbach G,Kiesmann M
Mon Jan 31 00:00:00 EST 2011
TDP-43 regulates its mRNA levels through a negative feedback loop.
The EMBO journal
Ayala YM,De Conti L,Avendaño-Vázquez SE,Dhir A,Romano M,D'Ambrogio A,Tollervey J,Ule J,Baralle M,Buratti E,Baralle FE
Wed Jan 19 00:00:00 EST 2011
FALS with Gly72Ser mutation in SOD1 gene: report of a family including the first autopsy case.
Journal of the neurological sciences
Kobayashi Z,Tsuchiya K,Kubodera T,Shibata N,Arai T,Miura H,Ishikawa C,Kondo H,Ishizu H,Akiyama H,Mizusawa H
Sat Jan 15 00:00:00 EST 2011
Identification of neuronal RNA targets of TDP-43-containing ribonucleoprotein complexes.
The Journal of biological chemistry
Sephton CF,Cenik C,Kucukural A,Dammer EB,Cenik B,Han Y,Dewey CM,Roth FP,Herz J,Peng J,Moore MJ,Yu G
Fri Jan 14 00:00:00 EST 2011
Identification of neuronal RNA targets of TDP-43-containing ribonucleoprotein complexes.
The Journal of biological chemistry
Sephton CF,Cenik C,Kucukural A,Dammer EB,Cenik B,Han Y,Dewey CM,Roth FP,Herz J,Peng J,Moore MJ,Yu G
Fri Jan 14 00:00:00 EST 2011
Identification of neuronal RNA targets of TDP-43-containing ribonucleoprotein complexes.
The Journal of biological chemistry
Sephton CF,Cenik C,Kucukural A,Dammer EB,Cenik B,Han Y,Dewey CM,Roth FP,Herz J,Peng J,Moore MJ,Yu G
Fri Jan 14 00:00:00 EST 2011
Biochemical, neuropathological, and neuroimaging characteristics of early-onset Alzheimer's disease due to a novel PSEN1 mutation.
Neuroscience letters
Ringman JM,Gylys KH,Medina LD,Fox M,Kepe V,Flores DL,Apostolova LG,Barrio JR,Small G,Silverman DH,Siu E,Cederbaum S,Hecimovic S,Malnar M,Chakraverty S,Goate AM,Bird TD,Leverenz JB
Mon Jan 10 00:00:00 EST 2011
Nuclear transport impairment of amyotrophic lateral sclerosis-linked mutations in FUS/TLS.
Annals of neurology
Ito D,Seki M,Tsunoda Y,Uchiyama H,Suzuki N
Sat Jan 01 00:00:00 EST 2011
Regulation of TDP-43 aggregation by phosphorylation and p62/SQSTM1.
Journal of neurochemistry
Brady OA,Meng P,Zheng Y,Mao Y,Hu F
Sat Jan 01 00:00:00 EST 2011
Regulation of TDP-43 aggregation by phosphorylation and p62/SQSTM1.
Journal of neurochemistry
Brady OA,Meng P,Zheng Y,Mao Y,Hu F
Sat Jan 01 00:00:00 EST 2011
The C-terminal TDP-43 fragments have a high aggregation propensity and harm neurons by a dominant-negative mechanism.
PloS one
Yang C,Tan W,Whittle C,Qiu L,Cao L,Akbarian S,Xu Z
Fri Dec 31 00:00:00 EST 2010
The C-terminal TDP-43 fragments have a high aggregation propensity and harm neurons by a dominant-negative mechanism.
PloS one
Yang C,Tan W,Whittle C,Qiu L,Cao L,Akbarian S,Xu Z
Fri Dec 31 00:00:00 EST 2010
Progranulin deficiency leads to enhanced cell vulnerability and TDP-43 translocation in primary neuronal cultures.
Brain research
Guo A,Tapia L,Bamji SX,Cynader MS,Jia W
Fri Dec 17 00:00:00 EST 2010
Does TDP-43 type confer a distinct pattern of atrophy in frontotemporal lobar degeneration?
Neurology
Whitwell JL,Jack CR,Parisi JE,Senjem ML,Knopman DS,Boeve BF,Rademakers R,Baker M,Petersen RC,Dickson DW,Josephs KA
Tue Dec 14 00:00:00 EST 2010
TDP-43 subtypes are associated with distinct atrophy patterns in frontotemporal dementia.
Neurology
Rohrer JD,Geser F,Zhou J,Gennatas ED,Sidhu M,Trojanowski JQ,Dearmond SJ,Miller BL,Seeley WW
Tue Dec 14 00:00:00 EST 2010
Exome sequencing reveals VCP mutations as a cause of familial ALS.
Neuron
Johnson JO,Mandrioli J,Benatar M,Abramzon Y,Van Deerlin VM,Trojanowski JQ,Gibbs JR,Brunetti M,Gronka S,Wuu J,Ding J,McCluskey L,Martinez-Lage M,Falcone D,Hernandez DG,Arepalli S,Chong S,Schymick JC,Rothstein J,Landi F,Wang YD,Calvo A,Mora G,Sabatelli M,Monsurrò MR,Battistini S,Salvi F,Spataro R,Sola P,Borghero G,Galassi G,Scholz SW,Taylor JP,Restagno G,Chiò A,Traynor BJ
Thu Dec 09 00:00:00 EST 2010
Neuropathologic classification of dementias: introduction.
Handbook of clinical neurology
Duyckaerts C
Tue Dec 07 00:00:00 EST 2010
Phosphoproteomic analysis reveals site-specific changes in GFAP and NDRG2 phosphorylation in frontotemporal lobar degeneration.
Journal of proteome research
Herskowitz JH,Seyfried NT,Duong DM,Xia Q,Rees HD,Gearing M,Peng J,Lah JJ,Levey AI
Fri Dec 03 00:00:00 EST 2010
TDP-43 physically interacts with amyotrophic lateral sclerosis-linked mutant CuZn superoxide dismutase.
Neurochemistry international
Higashi S,Tsuchiya Y,Araki T,Wada K,Kabuta T
Wed Dec 01 00:00:00 EST 2010
TDP-43 physically interacts with amyotrophic lateral sclerosis-linked mutant CuZn superoxide dismutase.
Neurochemistry international
Higashi S,Tsuchiya Y,Araki T,Wada K,Kabuta T
Wed Dec 01 00:00:00 EST 2010
Very early-onset frontotemporal dementia with no family history predicts underlying fused in sarcoma pathology.
Brain : a journal of neurology
Loy CT,McCusker E,Kril JJ,Kwok JB,Brooks WS,McCann H,Isaacs AM,Halliday GM
Wed Dec 01 00:00:00 EST 2010
TDP-43 proteinopathy in familial motor neurone disease with TARDBP A315T mutation: a case report.
Neuropathology and applied neurobiology
Cairns NJ,Perrin RJ,Schmidt RE,Gru A,Green KG,Carter D,Taylor-Reinwald L,Morris JC,Gitcho MA,Baloh RH
Wed Dec 01 00:00:00 EST 2010
Widespread occurrence of eosinophilic neuronal cytoplasmic inclusions in an asymptomatic adult: a novel ubiquitin-negative filamentous inclusion.
Neuropathology : official journal of the Japanese Society of Neuropathology
Mori F,Miki Y,Tanji K,Kusumi T,Kijima H,Wakabayashi K
Wed Dec 01 00:00:00 EST 2010
Pathologic evidence that the T188R mutation in PRNP is associated with prion disease.
Journal of neuropathology and experimental neurology
Tartaglia MC,Thai JN,See T,Kuo A,Harbaugh R,Raudabaugh B,Cali I,Sattavat M,Sanchez H,DeArmond SJ,Geschwind MD
Wed Dec 01 00:00:00 EST 2010
Age-dependent changes in TDP-43 levels in a mouse model of Alzheimer disease are linked to Aβ oligomers accumulation.
Molecular neurodegeneration
Caccamo A,Magrí A,Oddo S
Thu Nov 11 00:00:00 EST 2010
Extensive FUS-immunoreactive pathology in juvenile amyotrophic lateral sclerosis with basophilic inclusions.
Brain pathology (Zurich, Switzerland)
Huang EJ,Zhang J,Geser F,Trojanowski JQ,Strober JB,Dickson DW,Brown RH,Shapiro BE,Lomen-Hoerth C
Mon Nov 01 00:00:00 EDT 2010
Increased caspase activation and decreased TDP-43 solubility in progranulin knockout cortical cultures.
Journal of neurochemistry
Kleinberger G,Wils H,Ponsaerts P,Joris G,Timmermans JP,Van Broeckhoven C,Kumar-Singh S
Mon Nov 01 00:00:00 EDT 2010
Sigma nonopioid intracellular receptor 1 mutations cause frontotemporal lobar degeneration-motor neuron disease.
Annals of neurology
Luty AA,Kwok JB,Dobson-Stone C,Loy CT,Coupland KG,Karlström H,Sobow T,Tchorzewska J,Maruszak A,Barcikowska M,Panegyres PK,Zekanowski C,Brooks WS,Williams KL,Blair IP,Mather KA,Sachdev PS,Halliday GM,Schofield PR
Mon Nov 01 00:00:00 EDT 2010
Sigma nonopioid intracellular receptor 1 mutations cause frontotemporal lobar degeneration-motor neuron disease.
Annals of neurology
Luty AA,Kwok JB,Dobson-Stone C,Loy CT,Coupland KG,Karlström H,Sobow T,Tchorzewska J,Maruszak A,Barcikowska M,Panegyres PK,Zekanowski C,Brooks WS,Williams KL,Blair IP,Mather KA,Sachdev PS,Halliday GM,Schofield PR
Mon Nov 01 00:00:00 EDT 2010
Amyotrophic lateral sclerosis-associated proteins TDP-43 and FUS/TLS function in a common biochemical complex to co-regulate HDAC6 mRNA.
The Journal of biological chemistry
Kim SH,Shanware NP,Bowler MJ,Tibbetts RS
Fri Oct 29 00:00:00 EDT 2010
Amyotrophic lateral sclerosis-associated proteins TDP-43 and FUS/TLS function in a common biochemical complex to co-regulate HDAC6 mRNA.
The Journal of biological chemistry
Kim SH,Shanware NP,Bowler MJ,Tibbetts RS
Fri Oct 29 00:00:00 EDT 2010
Progranulin is neurotrophic in vivo and protects against a mutant TDP-43 induced axonopathy.
PloS one
Laird AS,Van Hoecke A,De Muynck L,Timmers M,Van den Bosch L,Van Damme P,Robberecht W
Wed Oct 13 00:00:00 EDT 2010
Tar DNA binding protein-43 (TDP-43) associates with stress granules: analysis of cultured cells and pathological brain tissue.
PloS one
Liu-Yesucevitz L,Bilgutay A,Zhang YJ,Vanderweyde T,Vanderwyde T,Citro A,Mehta T,Zaarur N,McKee A,Bowser R,Sherman M,Petrucelli L,Wolozin B
Mon Oct 11 00:00:00 EDT 2010
Pathological 43-kDa transactivation response DNA-binding protein in older adults with and without severe mental illness.
Archives of neurology
Geser F,Robinson JL,Malunda JA,Xie SX,Clark CM,Kwong LK,Moberg PJ,Moore EM,Van Deerlin VM,Lee VM,Arnold SE,Trojanowski JQ
Fri Oct 01 00:00:00 EDT 2010
Lack of shunt response in suspected idiopathic normal pressure hydrocephalus with Alzheimer disease pathology.
Annals of neurology
Hamilton R,Patel S,Lee EB,Jackson EM,Lopinto J,Arnold SE,Clark CM,Basil A,Shaw LM,Xie SX,Grady MS,Trojanowski JQ
Fri Oct 01 00:00:00 EDT 2010
Age-related motor neuron degeneration in DNA repair-deficient Ercc1 mice.
Acta neuropathologica
de Waard MC,van der Pluijm I,Zuiderveen Borgesius N,Comley LH,Haasdijk ED,Rijksen Y,Ridwan Y,Zondag G,Hoeijmakers JH,Elgersma Y,Gillingwater TH,Jaarsma D
Fri Oct 01 00:00:00 EDT 2010
Involvement of Onuf's nucleus in Machado-Joseph disease: a morphometric and immunohistochemical study.
Acta neuropathologica
Shimizu H,Yamada M,Toyoshima Y,Ikeuchi T,Onodera O,Takahashi H
Fri Oct 01 00:00:00 EDT 2010
Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice.
Proceedings of the National Academy of Sciences of the United States of America
Shan X,Chiang PM,Price DL,Wong PC
Tue Sep 14 00:00:00 EDT 2010
Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice.
Proceedings of the National Academy of Sciences of the United States of America
Shan X,Chiang PM,Price DL,Wong PC
Tue Sep 14 00:00:00 EDT 2010
Deletion of TDP-43 down-regulates Tbc1d1, a gene linked to obesity, and alters body fat metabolism.
Proceedings of the National Academy of Sciences of the United States of America
Chiang PM,Ling J,Jeong YH,Price DL,Aja SM,Wong PC
Tue Sep 14 00:00:00 EDT 2010
Increased neuronal Rab5 immunoreactive endosomes do not colocalize with TDP-43 in motor neuron disease.
Experimental neurology
Matej R,Botond G,László L,Kopitar-Jerala N,Rusina R,Budka H,Kovacs GG
Wed Sep 01 00:00:00 EDT 2010
Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS.
Nature
Elden AC,Kim HJ,Hart MP,Chen-Plotkin AS,Johnson BS,Fang X,Armakola M,Geser F,Greene R,Lu MM,Padmanabhan A,Clay-Falcone D,McCluskey L,Elman L,Juhr D,Gruber PJ,Rüb U,Auburger G,Trojanowski JQ,Lee VM,Van Deerlin VM,Bonini NM,Gitler AD
Thu Aug 26 00:00:00 EDT 2010
Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS.
Nature
Elden AC,Kim HJ,Hart MP,Chen-Plotkin AS,Johnson BS,Fang X,Armakola M,Geser F,Greene R,Lu MM,Padmanabhan A,Clay-Falcone D,McCluskey L,Elman L,Juhr D,Gruber PJ,Rüb U,Auburger G,Trojanowski JQ,Lee VM,Van Deerlin VM,Bonini NM,Gitler AD
Thu Aug 26 00:00:00 EDT 2010
Interaction with polyglutamine aggregates reveals a Q/N-rich domain in TDP-43.
The Journal of biological chemistry
Fuentealba RA,Udan M,Bell S,Wegorzewska I,Shao J,Diamond MI,Weihl CC,Baloh RH
Fri Aug 20 00:00:00 EDT 2010
Interaction with polyglutamine aggregates reveals a Q/N-rich domain in TDP-43.
The Journal of biological chemistry
Fuentealba RA,Udan M,Bell S,Wegorzewska I,Shao J,Diamond MI,Weihl CC,Baloh RH
Fri Aug 20 00:00:00 EDT 2010
TDP-43-mediated neuron loss in vivo requires RNA-binding activity.
PloS one
Voigt A,Herholz D,Fiesel FC,Kaur K,Müller D,Karsten P,Weber SS,Kahle PJ,Marquardt T,Schulz JB
Wed Aug 18 00:00:00 EDT 2010
Juvenile ALS with basophilic inclusions is a FUS proteinopathy with FUS mutations.
Neurology
Bäumer D,Hilton D,Paine SM,Turner MR,Lowe J,Talbot K,Ansorge O
Tue Aug 17 00:00:00 EDT 2010
Neurotoxic effects of TDP-43 overexpression in C. elegans.
Human molecular genetics
Ash PE,Zhang YJ,Roberts CM,Saldi T,Hutter H,Buratti E,Petrucelli L,Link CD
Sun Aug 15 00:00:00 EDT 2010
Neurotoxic effects of TDP-43 overexpression in C. elegans.
Human molecular genetics
Ash PE,Zhang YJ,Roberts CM,Saldi T,Hutter H,Buratti E,Petrucelli L,Link CD
Sun Aug 15 00:00:00 EDT 2010
Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Xu YF,Gendron TF,Zhang YJ,Lin WL,D'Alton S,Sheng H,Casey MC,Tong J,Knight J,Yu X,Rademakers R,Boylan K,Hutton M,McGowan E,Dickson DW,Lewis J,Petrucelli L
Wed Aug 11 00:00:00 EDT 2010
Elevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U.
The Journal of experimental medicine
Tsai KJ,Yang CH,Fang YH,Cho KH,Chien WL,Wang WT,Wu TW,Lin CP,Fu WM,Shen CK
Mon Aug 02 00:00:00 EDT 2010
Elevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U.
The Journal of experimental medicine
Tsai KJ,Yang CH,Fang YH,Cho KH,Chien WL,Wang WT,Wu TW,Lin CP,Fu WM,Shen CK
Mon Aug 02 00:00:00 EDT 2010
Abnormal TDP-43 expression is identified in the neocortex in cases of dementia pugilistica, but is mainly confined to the limbic system when identified in high and moderate stages of Alzheimer's disease.
Neuropathology : official journal of the Japanese Society of Neuropathology
King A,Sweeney F,Bodi I,Troakes C,Maekawa S,Al-Sarraj S
Sun Aug 01 00:00:00 EDT 2010
Fus gene mutations in familial and sporadic amyotrophic lateral sclerosis.
Muscle & nerve
Rademakers R,Stewart H,Dejesus-Hernandez M,Krieger C,Graff-Radford N,Fabros M,Briemberg H,Cashman N,Eisen A,Mackenzie IR
Sun Aug 01 00:00:00 EDT 2010
Pseudopolyneuritic form of ALS revisited: clinical and pathological heterogeneity.
Neuropathology : official journal of the Japanese Society of Neuropathology
Kobayashi Z,Tsuchiya K,Arai T,Yokota O,Watabiki S,Ishizu H,Akiyama H,Mizusawa H
Sun Aug 01 00:00:00 EDT 2010
A novel X-linked four-repeat tauopathy with Parkinsonism and spasticity.
Movement disorders : official journal of the Movement Disorder Society
Poorkaj P,Raskind WH,Leverenz JB,Matsushita M,Zabetian CP,Samii A,Kim S,Gazi N,Nutt JG,Wolff J,Yearout D,Greenup JL,Steinbart EJ,Bird TD
Fri Jul 30 00:00:00 EDT 2010
ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS.
Proceedings of the National Academy of Sciences of the United States of America
Ling SC,Albuquerque CP,Han JS,Lagier-Tourenne C,Tokunaga S,Zhou H,Cleveland DW
Tue Jul 27 00:00:00 EDT 2010
ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS.
Proceedings of the National Academy of Sciences of the United States of America
Ling SC,Albuquerque CP,Han JS,Lagier-Tourenne C,Tokunaga S,Zhou H,Cleveland DW
Tue Jul 27 00:00:00 EDT 2010
Anatomical differences between CBS-corticobasal degeneration and CBS-Alzheimer's disease.
Movement disorders : official journal of the Movement Disorder Society
Josephs KA,Whitwell JL,Boeve BF,Knopman DS,Petersen RC,Hu WT,Parisi JE,Dickson DW,Jack CR
Thu Jul 15 00:00:00 EDT 2010
Alterations in subcellular localization of TDP-43 immunoreactivity in the anterior horns in sporadic amyotrophic lateral sclerosis.
Neuroscience letters
Sasaki S,Takeda T,Shibata N,Kobayashi M
Mon Jul 05 00:00:00 EDT 2010
Sustained expression of TDP-43 and FUS in motor neurons in rodent's lifetime.
International journal of biological sciences
Huang C,Xia PY,Zhou H
Sun Jul 04 00:00:00 EDT 2010
Accelerated lipofuscinosis and ubiquitination in granulin knockout mice suggest a role for progranulin in successful aging.
The American journal of pathology
Ahmed Z,Sheng H,Xu YF,Lin WL,Innes AE,Gass J,Yu X,Wuertzer CA,Hou H,Chiba S,Yamanouchi K,Leissring M,Petrucelli L,Nishihara M,Hutton ML,McGowan E,Dickson DW,Lewis J
Thu Jul 01 00:00:00 EDT 2010
Caudate atrophy on MRI is a characteristic feature of FTLD-FUS.
European journal of neurology
Josephs KA,Whitwell JL,Parisi JE,Petersen RC,Boeve BF,Jack CR,Dickson DW
Thu Jul 01 00:00:00 EDT 2010
TDP-43 pathology in primary progressive aphasia and frontotemporal dementia with pathologic Alzheimer disease.
Acta neuropathologica
Bigio EH,Mishra M,Hatanpaa KJ,White CL,Johnson N,Rademaker A,Weitner BB,Deng HX,Dubner SD,Weintraub S,Mesulam M
Thu Jul 01 00:00:00 EDT 2010
Neuropathology of olfactory ensheathing cell transplantation into the brain of two amyotrophic lateral sclerosis (ALS) patients.
Brain pathology (Zurich, Switzerland)
Giordana MT,Grifoni S,Votta B,Magistrello M,Vercellino M,Pellerino A,Navone R,Valentini C,Calvo A,Chiò A
Thu Jul 01 00:00:00 EDT 2010
TDP-43 pathology in sporadic ALS occurs in motor neurons lacking the RNA editing enzyme ADAR2.
Acta neuropathologica
Aizawa H,Sawada J,Hideyama T,Yamashita T,Katayama T,Hasebe N,Kimura T,Yahara O,Kwak S
Thu Jul 01 00:00:00 EDT 2010
TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Ritson GP,Custer SK,Freibaum BD,Guinto JB,Geffel D,Moore J,Tang W,Winton MJ,Neumann M,Trojanowski JQ,Lee VM,Forman MS,Taylor JP
Wed Jun 02 00:00:00 EDT 2010
TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Ritson GP,Custer SK,Freibaum BD,Guinto JB,Geffel D,Moore J,Tang W,Winton MJ,Neumann M,Trojanowski JQ,Lee VM,Forman MS,Taylor JP
Wed Jun 02 00:00:00 EDT 2010
Nuclear import impairment causes cytoplasmic trans-activation response DNA-binding protein accumulation and is associated with frontotemporal lobar degeneration.
Brain : a journal of neurology
Nishimura AL,Zupunski V,Troakes C,Kathe C,Fratta P,Howell M,Gallo JM,Hortobágyi T,Shaw CE,Rogelj B
Tue Jun 01 00:00:00 EDT 2010
Nuclear import impairment causes cytoplasmic trans-activation response DNA-binding protein accumulation and is associated with frontotemporal lobar degeneration.
Brain : a journal of neurology
Nishimura AL,Zupunski V,Troakes C,Kathe C,Fratta P,Howell M,Gallo JM,Hortobágyi T,Shaw CE,Rogelj B
Tue Jun 01 00:00:00 EDT 2010
FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosis.
Annals of neurology
Deng HX,Zhai H,Bigio EH,Yan J,Fecto F,Ajroud K,Mishra M,Ajroud-Driss S,Heller S,Sufit R,Siddique N,Mugnaini E,Siddique T
Tue Jun 01 00:00:00 EDT 2010
FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosis.
Annals of neurology
Deng HX,Zhai H,Bigio EH,Yan J,Fecto F,Ajroud K,Mishra M,Ajroud-Driss S,Heller S,Sufit R,Siddique N,Mugnaini E,Siddique T
Tue Jun 01 00:00:00 EDT 2010
Relationship between Bunina bodies and TDP-43 inclusions in spinal anterior horn in amyotrophic lateral sclerosis.
Neuropathology and applied neurobiology
Mori F,Tanji K,Miki Y,Kakita A,Takahashi H,Wakabayashi K
Tue Jun 01 00:00:00 EDT 2010
FUS mutations in amyotrophic lateral sclerosis: clinical, pathological, neurophysiological and genetic analysis.
Journal of neurology, neurosurgery, and psychiatry
Blair IP,Williams KL,Warraich ST,Durnall JC,Thoeng AD,Manavis J,Blumbergs PC,Vucic S,Kiernan MC,Nicholson GA
Tue Jun 01 00:00:00 EDT 2010
TDP-43 M337V mutation in familial amyotrophic lateral sclerosis in Japan.
Internal medicine (Tokyo, Japan)
Tamaoka A,Arai M,Itokawa M,Arai T,Hasegawa M,Tsuchiya K,Takuma H,Tsuji H,Ishii A,Watanabe M,Takahashi Y,Goto J,Tsuji S,Akiyama H
Tue May 25 00:00:00 EDT 2010
Mutant superoxide dismutase 1 overexpression in NSC-34 cells: effect of trehalose on aggregation, TDP-43 localization and levels of co-expressed glycoproteins.
Neuroscience letters
Gomes C,Escrevente C,Costa J
Fri May 21 00:00:00 EDT 2010
Amyotrophic lateral sclerosis mutant vesicle-associated membrane protein-associated protein-B transgenic mice develop TAR-DNA-binding protein-43 pathology.
Neuroscience
Tudor EL,Galtrey CM,Perkinton MS,Lau KF,De Vos KJ,Mitchell JC,Ackerley S,Hortobágyi T,Vámos E,Leigh PN,Klasen C,McLoughlin DM,Shaw CE,Miller CC
Wed May 19 00:00:00 EDT 2010
Mutations of optineurin in amyotrophic lateral sclerosis.
Nature
Maruyama H,Morino H,Ito H,Izumi Y,Kato H,Watanabe Y,Kinoshita Y,Kamada M,Nodera H,Suzuki H,Komure O,Matsuura S,Kobatake K,Morimoto N,Abe K,Suzuki N,Aoki M,Kawata A,Hirai T,Kato T,Ogasawara K,Hirano A,Takumi T,Kusaka H,Hagiwara K,Kaji R,Kawakami H
Thu May 13 00:00:00 EDT 2010
TDP-43 and ubiquitinated cytoplasmic aggregates in sporadic ALS are low frequency and widely distributed in the lower motor neuron columns independent of disease spread.
Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
Bodansky A,Kim JM,Tempest L,Velagapudi A,Libby R,Ravits J
Mon May 03 00:00:00 EDT 2010
TDP-43 dimerizes in human cells in culture.
Cellular and molecular neurobiology
Shiina Y,Arima K,Tabunoki H,Satoh J
Sat May 01 00:00:00 EDT 2010
FUS-immunoreactive intranuclear inclusions in neurodegenerative disease.
Brain pathology (Zurich, Switzerland)
Woulfe J,Gray DA,Mackenzie IR
Sat May 01 00:00:00 EDT 2010
A mutation affecting the sodium/proton exchanger, SLC9A6, causes mental retardation with tau deposition.
Brain : a journal of neurology
Garbern JY,Neumann M,Trojanowski JQ,Lee VM,Feldman G,Norris JW,Friez MJ,Schwartz CE,Stevenson R,Sima AA
Sat May 01 00:00:00 EDT 2010
Efficient conditional and promoter-specific in vivo expression of cDNAs of choice by taking advantage of recombinase-mediated cassette exchange using FlEx gene traps.
Nucleic acids research
Schebelle L,Wolf C,Stribl C,Javaheri T,Schnütgen F,Ettinger A,Ivics Z,Hansen J,Ruiz P,von Melchner H,Wurst W,Floss T
Sat May 01 00:00:00 EDT 2010
Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone.
Human molecular genetics
Custer SK,Neumann M,Lu H,Wright AC,Taylor JP
Sat May 01 00:00:00 EDT 2010
Familial amyotrophic lateral sclerosis is associated with a mutation in D-amino acid oxidase.
Proceedings of the National Academy of Sciences of the United States of America
Mitchell J,Paul P,Chen HJ,Morris A,Payling M,Falchi M,Habgood J,Panoutsou S,Winkler S,Tisato V,Hajitou A,Smith B,Vance C,Shaw C,Mazarakis ND,de Belleroche J
Tue Apr 20 00:00:00 EDT 2010
Ubiquilin modifies TDP-43 toxicity in a Drosophila model of amyotrophic lateral sclerosis (ALS).
The Journal of biological chemistry
Hanson KA,Kim SH,Wassarman DA,Tibbetts RS
Fri Apr 09 00:00:00 EDT 2010
Ubiquilin modifies TDP-43 toxicity in a Drosophila model of amyotrophic lateral sclerosis (ALS).
The Journal of biological chemistry
Hanson KA,Kim SH,Wassarman DA,Tibbetts RS
Fri Apr 09 00:00:00 EDT 2010
Multiplex SILAC analysis of a cellular TDP-43 proteinopathy model reveals protein inclusions associated with SUMOylation and diverse polyubiquitin chains.
Molecular & cellular proteomics : MCP
Seyfried NT,Gozal YM,Dammer EB,Xia Q,Duong DM,Cheng D,Lah JJ,Levey AI,Peng J
Thu Apr 01 00:00:00 EDT 2010
Multiplex SILAC analysis of a cellular TDP-43 proteinopathy model reveals protein inclusions associated with SUMOylation and diverse polyubiquitin chains.
Molecular & cellular proteomics : MCP
Seyfried NT,Gozal YM,Dammer EB,Xia Q,Duong DM,Cheng D,Lah JJ,Levey AI,Peng J
Thu Apr 01 00:00:00 EDT 2010
FALS with FUS mutation in Japan, with early onset, rapid progress and basophilic inclusion.
Journal of human genetics
Suzuki N,Aoki M,Warita H,Kato M,Mizuno H,Shimakura N,Akiyama T,Furuya H,Hokonohara T,Iwaki A,Togashi S,Konno H,Itoyama Y
Thu Apr 01 00:00:00 EDT 2010
Olfactory epithelium amyloid-beta and paired helical filament-tau pathology in Alzheimer disease.
Annals of neurology
Arnold SE,Lee EB,Moberg PJ,Stutzbach L,Kazi H,Han LY,Lee VM,Trojanowski JQ
Thu Apr 01 00:00:00 EDT 2010
Survival in progressive supranuclear palsy and frontotemporal dementia.
Journal of neurology, neurosurgery, and psychiatry
Chiu WZ,Kaat LD,Seelaar H,Rosso SM,Boon AJ,Kamphorst W,van Swieten JC
Thu Apr 01 00:00:00 EDT 2010
Alteration of biochemical and pathological properties of TDP-43 protein by a lipid mediator, 15-deoxy-Delta(12,14)-prostaglandin J(2).
Experimental neurology
Zhang HX,Tanji K,Yoshida H,Hayakari M,Shibata T,Mori F,Uchida K,Wakabayashi K
Thu Apr 01 00:00:00 EDT 2010
Alteration of biochemical and pathological properties of TDP-43 protein by a lipid mediator, 15-deoxy-Delta(12,14)-prostaglandin J(2).
Experimental neurology
Zhang HX,Tanji K,Yoshida H,Hayakari M,Shibata T,Mori F,Uchida K,Wakabayashi K
Thu Apr 01 00:00:00 EDT 2010
Alzheimer's disease phenotypes and genotypes associated with mutations in presenilin 2.
Brain : a journal of neurology
Jayadev S,Leverenz JB,Steinbart E,Stahl J,Klunk W,Yu CE,Bird TD
Thu Apr 01 00:00:00 EDT 2010
Sporadic amyotrophic lateral sclerosis with pallido-nigro-luysian degeneration: a TDP-43 immunohistochemical study.
Neuropathology : official journal of the Japanese Society of Neuropathology
Miki Y,Mori F,Nunomura J,Ookawa K,Yajima N,Yagihashi S,Wakabayashi K
Thu Apr 01 00:00:00 EDT 2010
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.
PLoS genetics
Zhou H,Huang C,Chen H,Wang D,Landel CP,Xia PY,Bowser R,Liu YJ,Xia XG
Fri Mar 26 00:00:00 EDT 2010
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.
PLoS genetics
Zhou H,Huang C,Chen H,Wang D,Landel CP,Xia PY,Bowser R,Liu YJ,Xia XG
Fri Mar 26 00:00:00 EDT 2010
Loss of ALS2/Alsin exacerbates motor dysfunction in a SOD1-expressing mouse ALS model by disturbing endolysosomal trafficking.
PloS one
Hadano S,Otomo A,Kunita R,Suzuki-Utsunomiya K,Akatsuka A,Koike M,Aoki M,Uchiyama Y,Itoyama Y,Ikeda JE
Mon Mar 22 00:00:00 EDT 2010
Synergistic effect between proteasome and autophagosome in the clearance of polyubiquitinated TDP-43.
Journal of neuroscience research
Urushitani M,Sato T,Bamba H,Hisa Y,Tooyama I
Mon Mar 01 00:00:00 EST 2010
Sporadic corticobasal syndrome due to FTLD-TDP.
Acta neuropathologica
Tartaglia MC,Sidhu M,Laluz V,Racine C,Rabinovici GD,Creighton K,Karydas A,Rademakers R,Huang EJ,Miller BL,DeArmond SJ,Seeley WW
Mon Mar 01 00:00:00 EST 2010
TDP-43 redistribution is an early event in sporadic amyotrophic lateral sclerosis.
Brain pathology (Zurich, Switzerland)
Giordana MT,Piccinini M,Grifoni S,De Marco G,Vercellino M,Magistrello M,Pellerino A,Buccinnà B,Lupino E,Rinaudo MT
Mon Mar 01 00:00:00 EST 2010
TDP-43 redistribution is an early event in sporadic amyotrophic lateral sclerosis.
Brain pathology (Zurich, Switzerland)
Giordana MT,Piccinini M,Grifoni S,De Marco G,Vercellino M,Magistrello M,Pellerino A,Buccinnà B,Lupino E,Rinaudo MT
Mon Mar 01 00:00:00 EST 2010
TDP-43 is a developmentally regulated protein essential for early embryonic development.
The Journal of biological chemistry
Sephton CF,Good SK,Atkin S,Dewey CM,Mayer P,Herz J,Yu G
Fri Feb 26 00:00:00 EST 2010
TDP-43 is a developmentally regulated protein essential for early embryonic development.
The Journal of biological chemistry
Sephton CF,Good SK,Atkin S,Dewey CM,Mayer P,Herz J,Yu G
Fri Feb 26 00:00:00 EST 2010
TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration.
Proceedings of the National Academy of Sciences of the United States of America
Wils H,Kleinberger G,Janssens J,Pereson S,Joris G,Cuijt I,Smits V,Ceuterick-de Groote C,Van Broeckhoven C,Kumar-Singh S
Tue Feb 23 00:00:00 EST 2010
Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo.
Human molecular genetics
Kabashi E,Lin L,Tradewell ML,Dion PA,Bercier V,Bourgouin P,Rochefort D,Bel Hadj S,Durham HD,Vande Velde C,Rouleau GA,Drapeau P
Mon Feb 15 00:00:00 EST 2010
Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo.
Human molecular genetics
Kabashi E,Lin L,Tradewell ML,Dion PA,Bercier V,Bourgouin P,Rochefort D,Bel Hadj S,Durham HD,Vande Velde C,Rouleau GA,Drapeau P
Mon Feb 15 00:00:00 EST 2010
Global analysis of TDP-43 interacting proteins reveals strong association with RNA splicing and translation machinery.
Journal of proteome research
Freibaum BD,Chitta RK,High AA,Taylor JP
Fri Feb 05 00:00:00 EST 2010
Global analysis of TDP-43 interacting proteins reveals strong association with RNA splicing and translation machinery.
Journal of proteome research
Freibaum BD,Chitta RK,High AA,Taylor JP
Fri Feb 05 00:00:00 EST 2010
Alzheimer disease-like phenotype associated with the c.154delA mutation in progranulin.
Archives of neurology
Kelley BJ,Haidar W,Boeve BF,Baker M,Shiung M,Knopman DS,Rademakers R,Hutton M,Adamson J,Kuntz KM,Dickson DW,Parisi JE,Smith GE,Petersen RC
Mon Feb 01 00:00:00 EST 2010
Neuropathological heterogeneity in frontotemporal lobar degeneration with TDP-43 proteinopathy: a quantitative study of 94 cases using principal components analysis.
Journal of neural transmission (Vienna, Austria : 1996)
Armstrong RA,Ellis W,Hamilton RL,Mackenzie IR,Hedreen J,Gearing M,Montine T,Vonsattel JP,Head E,Lieberman AP,Cairns NJ
Mon Feb 01 00:00:00 EST 2010
Exaggerated inflammation, impaired host defense, and neuropathology in progranulin-deficient mice.
The Journal of experimental medicine
Yin F,Banerjee R,Thomas B,Zhou P,Qian L,Jia T,Ma X,Ma Y,Iadecola C,Beal MF,Nathan C,Ding A
Mon Jan 18 00:00:00 EST 2010
Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Barmada SJ,Skibinski G,Korb E,Rao EJ,Wu JY,Finkbeiner S
Wed Jan 13 00:00:00 EST 2010
Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Barmada SJ,Skibinski G,Korb E,Rao EJ,Wu JY,Finkbeiner S
Wed Jan 13 00:00:00 EST 2010
Knockdown of transactive response DNA-binding protein (TDP-43) downregulates histone deacetylase 6.
The EMBO journal
Fiesel FC,Voigt A,Weber SS,Van den Haute C,Waldenmaier A,Görner K,Walter M,Anderson ML,Kern JV,Rasse TM,Schmidt T,Springer W,Kirchner R,Bonin M,Neumann M,Baekelandt V,Alunni-Fabbroni M,Schulz JB,Kahle PJ
Wed Jan 06 00:00:00 EST 2010
In vivo amyloid imaging in autopsy-confirmed Parkinson disease with dementia.
Neurology
Burack MA,Hartlein J,Flores HP,Taylor-Reinwald L,Perlmutter JS,Cairns NJ
Tue Jan 05 00:00:00 EST 2010
Modeling the association between 43 different clinical and pathological variables and the severity of cognitive impairment in a large autopsy cohort of elderly persons.
Brain pathology (Zurich, Switzerland)
Nelson PT,Abner EL,Schmitt FA,Kryscio RJ,Jicha GA,Smith CD,Davis DG,Poduska JW,Patel E,Mendiondo MS,Markesbery WR
Fri Jan 01 00:00:00 EST 2010
Characterization of alternative isoforms and inclusion body of the TAR DNA-binding protein-43.
The Journal of biological chemistry
Nishimoto Y,Ito D,Yagi T,Nihei Y,Tsunoda Y,Suzuki N
Fri Jan 01 00:00:00 EST 2010
Characterization of alternative isoforms and inclusion body of the TAR DNA-binding protein-43.
The Journal of biological chemistry
Nishimoto Y,Ito D,Yagi T,Nihei Y,Tsunoda Y,Suzuki N
Fri Jan 01 00:00:00 EST 2010
Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases.
Brain : a journal of neurology
Hodges JR,Mitchell J,Dawson K,Spillantini MG,Xuereb JH,McMonagle P,Nestor PJ,Patterson K
Fri Jan 01 00:00:00 EST 2010
Brain progranulin expression in GRN-associated frontotemporal lobar degeneration.
Acta neuropathologica
Chen-Plotkin AS,Xiao J,Geser F,Martinez-Lage M,Grossman M,Unger T,Wood EM,Van Deerlin VM,Trojanowski JQ,Lee VM
Fri Jan 01 00:00:00 EST 2010
Axonal ligation induces transient redistribution of TDP-43 in brainstem motor neurons.
Neuroscience
Sato T,Takeuchi S,Saito A,Ding W,Bamba H,Matsuura H,Hisa Y,Tooyama I,Urushitani M
Tue Dec 29 00:00:00 EST 2009
Axonal ligation induces transient redistribution of TDP-43 in brainstem motor neurons.
Neuroscience
Sato T,Takeuchi S,Saito A,Ding W,Bamba H,Matsuura H,Hisa Y,Tooyama I,Urushitani M
Tue Dec 29 00:00:00 EST 2009
Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease.
The Journal of cell biology
Ju JS,Fuentealba RA,Miller SE,Jackson E,Piwnica-Worms D,Baloh RH,Weihl CC
Mon Dec 14 00:00:00 EST 2009
Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease.
The Journal of cell biology
Ju JS,Fuentealba RA,Miller SE,Jackson E,Piwnica-Worms D,Baloh RH,Weihl CC
Mon Dec 14 00:00:00 EST 2009
Tar DNA binding protein of 43 kDa (TDP-43), 14-3-3 proteins and copper/zinc superoxide dismutase (SOD1) interact to modulate NFL mRNA stability. Implications for altered RNA processing in amyotrophic lateral sclerosis (ALS).
Brain research
Volkening K,Leystra-Lantz C,Yang W,Jaffee H,Strong MJ
Fri Dec 11 00:00:00 EST 2009
Tar DNA binding protein of 43 kDa (TDP-43), 14-3-3 proteins and copper/zinc superoxide dismutase (SOD1) interact to modulate NFL mRNA stability. Implications for altered RNA processing in amyotrophic lateral sclerosis (ALS).
Brain research
Volkening K,Leystra-Lantz C,Yang W,Jaffee H,Strong MJ
Fri Dec 11 00:00:00 EST 2009
Tar DNA binding protein of 43 kDa (TDP-43), 14-3-3 proteins and copper/zinc superoxide dismutase (SOD1) interact to modulate NFL mRNA stability. Implications for altered RNA processing in amyotrophic lateral sclerosis (ALS).
Brain research
Volkening K,Leystra-Lantz C,Yang W,Jaffee H,Strong MJ
Fri Dec 11 00:00:00 EST 2009
Absence of Pittsburgh compound B detection of cerebral amyloid beta in a patient with clinical, cognitive, and cerebrospinal fluid markers of Alzheimer disease: a case report.
Archives of neurology
Cairns NJ,Ikonomovic MD,Benzinger T,Storandt M,Fagan AM,Shah AR,Reinwald LT,Carter D,Felton A,Holtzman DM,Mintun MA,Klunk WE,Morris JC
Tue Dec 01 00:00:00 EST 2009
Transactive response DNA-binding protein 43 burden in familial Alzheimer disease and Down syndrome.
Archives of neurology
Lippa CF,Rosso AL,Stutzbach LD,Neumann M,Lee VM,Trojanowski JQ
Tue Dec 01 00:00:00 EST 2009
Sporadic amyotrophic lateral sclerosis: Widespread multisystem degeneration with TDP-43 pathology in a patient after long-term survival on a respirator.
Neuropathology : official journal of the Japanese Society of Neuropathology
Nishihira Y,Tan CF,Toyoshima Y,Yonemochi Y,Kondo H,Nakajima T,Takahashi H
Tue Dec 01 00:00:00 EST 2009
TDP-43 is consistently co-localized with ubiquitinated inclusions in sporadic and Guam amyotrophic lateral sclerosis but not in familial amyotrophic lateral sclerosis with and without SOD1 mutations.
Neuropathology : official journal of the Japanese Society of Neuropathology
Maekawa S,Leigh PN,King A,Jones E,Steele JC,Bodi I,Shaw CE,Hortobagyi T,Al-Sarraj S
Tue Dec 01 00:00:00 EST 2009
Cytosolic TDP-43 expression following axotomy is associated with caspase 3 activation in NFL-/- mice: support for a role for TDP-43 in the physiological response to neuronal injury.
Brain research
Moisse K,Mepham J,Volkening K,Welch I,Hill T,Strong MJ
Tue Nov 03 00:00:00 EST 2009
TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration.
Proceedings of the National Academy of Sciences of the United States of America
Wegorzewska I,Bell S,Cairns NJ,Miller TM,Baloh RH
Tue Nov 03 00:00:00 EST 2009
TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration.
Proceedings of the National Academy of Sciences of the United States of America
Wegorzewska I,Bell S,Cairns NJ,Miller TM,Baloh RH
Tue Nov 03 00:00:00 EST 2009
A new subtype of frontotemporal lobar degeneration with FUS pathology.
Brain : a journal of neurology
Neumann M,Rademakers R,Roeber S,Baker M,Kretzschmar HA,Mackenzie IR
Sun Nov 01 00:00:00 EDT 2009
Transactivation response DNA-binding protein 43 microvasculopathy in frontotemporal degeneration and familial Lewy body disease.
Journal of neuropathology and experimental neurology
Lin WL,Castanedes-Casey M,Dickson DW
Sun Nov 01 00:00:00 EDT 2009
Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease.
Acta neuropathologica
Neumann M,Roeber S,Kretzschmar HA,Rademakers R,Baker M,Mackenzie IR
Sun Nov 01 00:00:00 EDT 2009
Plasma phosphorylated-TDP-43 protein levels correlate with brain pathology in frontotemporal lobar degeneration.
Acta neuropathologica
Foulds PG,Davidson Y,Mishra M,Hobson DJ,Humphreys KM,Taylor M,Johnson N,Weintraub S,Akiyama H,Arai T,Hasegawa M,Bigio EH,Benson FE,Allsop D,Mann DM
Sun Nov 01 00:00:00 EDT 2009
TARDBP 3'-UTR variant in autopsy-confirmed frontotemporal lobar degeneration with TDP-43 proteinopathy.
Acta neuropathologica
Gitcho MA,Bigio EH,Mishra M,Johnson N,Weintraub S,Mesulam M,Rademakers R,Chakraverty S,Cruchaga C,Morris JC,Goate AM,Cairns NJ
Sun Nov 01 00:00:00 EDT 2009
TDP-43 is recruited to stress granules in conditions of oxidative insult.
Journal of neurochemistry
Colombrita C,Zennaro E,Fallini C,Weber M,Sommacal A,Buratti E,Silani V,Ratti A
Sun Nov 01 00:00:00 EDT 2009
TDP-43 is recruited to stress granules in conditions of oxidative insult.
Journal of neurochemistry
Colombrita C,Zennaro E,Fallini C,Weber M,Sommacal A,Buratti E,Silani V,Ratti A
Sun Nov 01 00:00:00 EDT 2009
Decreased cystatin C immunoreactivity in spinal motor neurons and astrocytes in amyotrophic lateral sclerosis.
Journal of neuropathology and experimental neurology
Mori F,Tanji K,Miki Y,Wakabayashi K
Sun Nov 01 00:00:00 EDT 2009
Accumulation of TDP-43 and alpha-actin in an amyotrophic lateral sclerosis patient with the K17I ANG mutation.
Acta neuropathologica
Seilhean D,Cazeneuve C,Thuriès V,Russaouen O,Millecamps S,Salachas F,Meininger V,Leguern E,Duyckaerts C
Thu Oct 01 00:00:00 EDT 2009
Selective occurrence of TDP-43-immunoreactive inclusions in the lower motor neurons in Machado-Joseph disease.
Acta neuropathologica
Tan CF,Yamada M,Toyoshima Y,Yokoseki A,Miki Y,Hoshi Y,Kaneko H,Ikeuchi T,Onodera O,Kakita A,Takahashi H
Thu Oct 01 00:00:00 EDT 2009
Regional distribution of TDP-43 inclusions in Alzheimer disease (AD) brains: their relation to AD common pathology.
Neuropathology : official journal of the Japanese Society of Neuropathology
Kadokura A,Yamazaki T,Lemere CA,Takatama M,Okamoto K
Thu Oct 01 00:00:00 EDT 2009
Chemical manipulation of hsp70 ATPase activity regulates tau stability.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Jinwal UK,Miyata Y,Koren J,Jones JR,Trotter JH,Chang L,O'Leary J,Morgan D,Lee DC,Shults CL,Rousaki A,Weeber EJ,Zuiderweg ER,Gestwicki JE,Dickey CA
Wed Sep 30 00:00:00 EDT 2009
Phosphorylation-dependent TDP-43 antibody detects intraneuronal dot-like structures showing morphological characters of granulovacuolar degeneration.
Neuroscience letters
Kadokura A,Yamazaki T,Kakuda S,Makioka K,Lemere CA,Fujita Y,Takatama M,Okamoto K
Tue Sep 29 00:00:00 EDT 2009
Phosphorylation-dependent TDP-43 antibody detects intraneuronal dot-like structures showing morphological characters of granulovacuolar degeneration.
Neuroscience letters
Kadokura A,Yamazaki T,Kakuda S,Makioka K,Lemere CA,Fujita Y,Takatama M,Okamoto K
Tue Sep 29 00:00:00 EDT 2009
Frontotemporal dementia and amyotrophic lateral sclerosis-associated disease protein TDP-43 promotes dendritic branching.
Molecular brain
Lu Y,Ferris J,Gao FB
Fri Sep 25 00:00:00 EDT 2009
TARDBP variation associated with frontotemporal dementia, supranuclear gaze palsy, and chorea.
Movement disorders : official journal of the Movement Disorder Society
Kovacs GG,Murrell JR,Horvath S,Haraszti L,Majtenyi K,Molnar MJ,Budka H,Ghetti B,Spina S
Tue Sep 15 00:00:00 EDT 2009
Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43.
Human molecular genetics
Nonaka T,Kametani F,Arai T,Akiyama H,Hasegawa M
Tue Sep 15 00:00:00 EDT 2009
Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43.
Human molecular genetics
Nonaka T,Kametani F,Arai T,Akiyama H,Hasegawa M
Tue Sep 15 00:00:00 EDT 2009
Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43.
Human molecular genetics
Nonaka T,Kametani F,Arai T,Akiyama H,Hasegawa M
Tue Sep 15 00:00:00 EDT 2009
TDP-43 in ubiquitinated inclusions in the inferior olives in frontotemporal lobar degeneration and in other neurodegenerative diseases: a degenerative process distinct from normal ageing.
Acta neuropathologica
Davidson Y,Amin H,Kelley T,Shi J,Tian J,Kumaran R,Lashley T,Lees AJ,DuPlessis D,Neary D,Snowden J,Akiyama H,Arai T,Hasegawa M,Bandopadhyay R,Sikkink S,Pickering-Brown S,Mann DM
Tue Sep 01 00:00:00 EDT 2009
TDP-43 localizes in mRNA transcription and processing sites in mammalian neurons.
Journal of structural biology
Casafont I,Bengoechea R,Tapia O,Berciano MT,Lafarga M
Tue Sep 01 00:00:00 EDT 2009
TDP-43 localizes in mRNA transcription and processing sites in mammalian neurons.
Journal of structural biology
Casafont I,Bengoechea R,Tapia O,Berciano MT,Lafarga M
Tue Sep 01 00:00:00 EDT 2009
Evaluation of subcortical pathology and clinical correlations in FTLD-U subtypes.
Acta neuropathologica
Josephs KA,Stroh A,Dugger B,Dickson DW
Tue Sep 01 00:00:00 EDT 2009
Abnormal localization of leucine-rich repeat kinase 2 to the endosomal-lysosomal compartment in lewy body disease.
Journal of neuropathology and experimental neurology
Higashi S,Moore DJ,Yamamoto R,Minegishi M,Sato K,Togo T,Katsuse O,Uchikado H,Furukawa Y,Hino H,Kosaka K,Emson PC,Wada K,Dawson VL,Dawson TM,Arai H,Iseki E
Tue Sep 01 00:00:00 EDT 2009
Clinical heterogeneity in 3 unrelated families linked to VCP p.Arg159His.
Neurology
van der Zee J,Pirici D,Van Langenhove T,Engelborghs S,Vandenberghe R,Hoffmann M,Pusswald G,Van den Broeck M,Peeters K,Mattheijssens M,Martin JJ,De Deyn PP,Cruts M,Haubenberger D,Kumar-Singh S,Zimprich A,Van Broeckhoven C
Tue Aug 25 00:00:00 EDT 2009
TDP-43 depletion induces neuronal cell damage through dysregulation of Rho family GTPases.
The Journal of biological chemistry
Iguchi Y,Katsuno M,Niwa JI,Yamada SI,Sone J,Waza M,Adachi H,Tanaka F,Nagata KI,Arimura N,Watanabe T,Kaibuchi K,Sobue G
Fri Aug 14 00:00:00 EDT 2009
Proteolytic processing of TAR DNA binding protein-43 by caspases produces C-terminal fragments with disease defining properties independent of progranulin.
Journal of neurochemistry
Dormann D,Capell A,Carlson AM,Shankaran SS,Rodde R,Neumann M,Kremmer E,Matsuwaki T,Yamanouchi K,Nishihara M,Haass C
Sat Aug 01 00:00:00 EDT 2009
Proteolytic processing of TAR DNA binding protein-43 by caspases produces C-terminal fragments with disease defining properties independent of progranulin.
Journal of neurochemistry
Dormann D,Capell A,Carlson AM,Shankaran SS,Rodde R,Neumann M,Kremmer E,Matsuwaki T,Yamanouchi K,Nishihara M,Haass C
Sat Aug 01 00:00:00 EDT 2009
Frontotemporal lobar degeneration with ubiquitinated tau-negative inclusions and additional alpha-synuclein pathology but also unusual cerebellar ubiquitinated p62-positive, TDP-43-negative inclusions.
Neuropathology : official journal of the Japanese Society of Neuropathology
King A,Al-Sarraj S,Shaw C
Sat Aug 01 00:00:00 EDT 2009
The dorsal raphe nucleus shows phospho-tau neurofibrillary changes before the transentorhinal region in Alzheimer's disease. A precocious onset?
Neuropathology and applied neurobiology
Grinberg LT,Rüb U,Ferretti RE,Nitrini R,Farfel JM,Polichiso L,Gierga K,Jacob-Filho W,Heinsen H
Sat Aug 01 00:00:00 EDT 2009
TDP-43 pathology in familial British dementia.
Acta neuropathologica
Schwab C,Arai T,Hasegawa M,Akiyama H,Yu S,McGeer PL
Sat Aug 01 00:00:00 EDT 2009
No Lewy pathology in monkeys with over 10 years of severe MPTP Parkinsonism.
Movement disorders : official journal of the Movement Disorder Society
Halliday G,Herrero MT,Murphy K,McCann H,Ros-Bernal F,Barcia C,Mori H,Blesa FJ,Obeso JA
Thu Jul 30 00:00:00 EDT 2009
Deep brain stimulation in dystonia: sonographic monitoring of electrode placement into the globus pallidus internus.
Movement disorders : official journal of the Movement Disorder Society
Walter U,Wolters A,Wittstock M,Benecke R,Schroeder HW,Müller JU
Thu Jul 30 00:00:00 EDT 2009
Methylene blue and dimebon inhibit aggregation of TDP-43 in cellular models.
FEBS letters
Yamashita M,Nonaka T,Arai T,Kametani F,Buchman VL,Ninkina N,Bachurin SO,Akiyama H,Goedert M,Hasegawa M
Tue Jul 21 00:00:00 EDT 2009
Methylene blue and dimebon inhibit aggregation of TDP-43 in cellular models.
FEBS letters
Yamashita M,Nonaka T,Arai T,Kametani F,Buchman VL,Ninkina N,Bachurin SO,Akiyama H,Goedert M,Hasegawa M
Tue Jul 21 00:00:00 EDT 2009
Mislocalization of TDP-43 in the G93A mutant SOD1 transgenic mouse model of ALS.
Neuroscience letters
Shan X,Vocadlo D,Krieger C
Fri Jul 17 00:00:00 EDT 2009
Mislocalization of TDP-43 in the G93A mutant SOD1 transgenic mouse model of ALS.
Neuroscience letters
Shan X,Vocadlo D,Krieger C
Fri Jul 17 00:00:00 EDT 2009
Sarcoplasmic redistribution of nuclear TDP-43 in inclusion body myositis.
Muscle & nerve
Salajegheh M,Pinkus JL,Taylor JP,Amato AA,Nazareno R,Baloh RH,Greenberg SA
Wed Jul 01 00:00:00 EDT 2009
Sarcoplasmic redistribution of nuclear TDP-43 in inclusion body myositis.
Muscle & nerve
Salajegheh M,Pinkus JL,Taylor JP,Amato AA,Nazareno R,Baloh RH,Greenberg SA
Wed Jul 01 00:00:00 EDT 2009
Hippocampal sclerosis with four-repeat tau-positive round inclusions in the dentate gyrus: a new type of four-repeat tauopathy.
Acta neuropathologica
Miki Y,Mori F,Hori E,Kaimori M,Wakabayashi K
Mon Jun 01 00:00:00 EDT 2009
Neuropathological investigation of regions responsible for semantic aphasia in frontotemporal lobar degeneration.
Dementia and geriatric cognitive disorders
Yamamoto R,Iseki E,Higashi S,Murayama N,Minegishi M,Sato K,Hino H,Fujisawa K,Kosaka K,Togo T,Katsuse O,Uchikado H,Furukawa Y,Yoshida M,Hashizume Y,Arai H
Thu May 28 00:00:00 EDT 2009
Prominent phenotypic variability associated with mutations in Progranulin.
Neurobiology of aging
Kelley BJ,Haidar W,Boeve BF,Baker M,Graff-Radford NR,Krefft T,Frank AR,Jack CR,Shiung M,Knopman DS,Josephs KA,Parashos SA,Rademakers R,Hutton M,Pickering-Brown S,Adamson J,Kuntz KM,Dickson DW,Parisi JE,Smith GE,Ivnik RJ,Petersen RC
Fri May 01 00:00:00 EDT 2009
VCP mutations causing frontotemporal lobar degeneration disrupt localization of TDP-43 and induce cell death.
The Journal of biological chemistry
Gitcho MA,Strider J,Carter D,Taylor-Reinwald L,Forman MS,Goate AM,Cairns NJ
Fri May 01 00:00:00 EDT 2009
VCP mutations causing frontotemporal lobar degeneration disrupt localization of TDP-43 and induce cell death.
The Journal of biological chemistry
Gitcho MA,Strider J,Carter D,Taylor-Reinwald L,Forman MS,Goate AM,Cairns NJ
Fri May 01 00:00:00 EDT 2009
Pallidonigral TDP-43 pathology in Perry syndrome.
Parkinsonism & related disorders
Wider C,Dickson DW,Stoessl AJ,Tsuboi Y,Chapon F,Gutmann L,Lechevalier B,Calne DB,Personett DA,Hulihan M,Kachergus J,Rademakers R,Baker MC,Grantier LL,Sujith OK,Brown L,Calne S,Farrer MJ,Wszolek ZK
Fri May 01 00:00:00 EDT 2009
Pallidonigral TDP-43 pathology in Perry syndrome.
Parkinsonism & related disorders
Wider C,Dickson DW,Stoessl AJ,Tsuboi Y,Chapon F,Gutmann L,Lechevalier B,Calne DB,Personett DA,Hulihan M,Kachergus J,Rademakers R,Baker MC,Grantier LL,Sujith OK,Brown L,Calne S,Farrer MJ,Wszolek ZK
Fri May 01 00:00:00 EDT 2009
Ubiquitin associated protein 1 is a risk factor for frontotemporal lobar degeneration.
Neurobiology of aging
Rollinson S,Rizzu P,Sikkink S,Baker M,Halliwell N,Snowden J,Traynor BJ,Ruano D,Cairns N,Rohrer JD,Mead S,Collinge J,Rossor M,Akay E,Guerreiro R,Rademakers R,Morrison KE,Pastor P,Alonso E,Martinez-Lage P,Graff-Radford N,Neary D,Heutink P,Mann DM,Van Swieten J,Pickering-Brown SM
Wed Apr 01 00:00:00 EDT 2009
TDP-43 neuropathology is similar in sporadic amyotrophic lateral sclerosis with or without TDP-43 mutations.
Neuropathology and applied neurobiology
Pamphlett R,Luquin N,McLean C,Jew SK,Adams L
Wed Apr 01 00:00:00 EDT 2009
Mimicking aspects of frontotemporal lobar degeneration and Lou Gehrig's disease in rats via TDP-43 overexpression.
Molecular therapy : the journal of the American Society of Gene Therapy
Tatom JB,Wang DB,Dayton RD,Skalli O,Hutton ML,Dickson DW,Klein RL
Wed Apr 01 00:00:00 EDT 2009
Mimicking aspects of frontotemporal lobar degeneration and Lou Gehrig's disease in rats via TDP-43 overexpression.
Molecular therapy : the journal of the American Society of Gene Therapy
Tatom JB,Wang DB,Dayton RD,Skalli O,Hutton ML,Dickson DW,Klein RL
Wed Apr 01 00:00:00 EDT 2009
Expression of TDP-43 C-terminal Fragments in Vitro Recapitulates Pathological Features of TDP-43 Proteinopathies.
The Journal of biological chemistry
Igaz LM,Kwong LK,Chen-Plotkin A,Winton MJ,Unger TL,Xu Y,Neumann M,Trojanowski JQ,Lee VM
Fri Mar 27 00:00:00 EDT 2009
Expression of TDP-43 C-terminal Fragments in Vitro Recapitulates Pathological Features of TDP-43 Proteinopathies.
The Journal of biological chemistry
Igaz LM,Kwong LK,Chen-Plotkin A,Winton MJ,Unger TL,Xu Y,Neumann M,Trojanowski JQ,Lee VM
Fri Mar 27 00:00:00 EDT 2009
Expression of TDP-43 C-terminal Fragments in Vitro Recapitulates Pathological Features of TDP-43 Proteinopathies.
The Journal of biological chemistry
Igaz LM,Kwong LK,Chen-Plotkin A,Winton MJ,Unger TL,Xu Y,Neumann M,Trojanowski JQ,Lee VM
Fri Mar 27 00:00:00 EDT 2009
Potentiation of amyotrophic lateral sclerosis (ALS)-associated TDP-43 aggregation by the proteasome-targeting factor, ubiquilin 1.
The Journal of biological chemistry
Kim SH,Shi Y,Hanson KA,Williams LM,Sakasai R,Bowler MJ,Tibbetts RS
Fri Mar 20 00:00:00 EDT 2009
Potentiation of amyotrophic lateral sclerosis (ALS)-associated TDP-43 aggregation by the proteasome-targeting factor, ubiquilin 1.
The Journal of biological chemistry
Kim SH,Shi Y,Hanson KA,Williams LM,Sakasai R,Bowler MJ,Tibbetts RS
Fri Mar 20 00:00:00 EDT 2009
Potentiation of amyotrophic lateral sclerosis (ALS)-associated TDP-43 aggregation by the proteasome-targeting factor, ubiquilin 1.
The Journal of biological chemistry
Kim SH,Shi Y,Hanson KA,Williams LM,Sakasai R,Bowler MJ,Tibbetts RS
Fri Mar 20 00:00:00 EDT 2009
Inflammatory myopathies with mitochondrial pathology and protein aggregates.
Journal of the neurological sciences
Temiz P,Weihl CC,Pestronk A
Sun Mar 15 00:00:00 EDT 2009
TDP-43 is not present in brain tissue of patients with schizophrenia.
Schizophrenia research
Mateen FJ,Josephs KA
Sun Mar 01 00:00:00 EST 2009
Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6.
Science (New York, N.Y.)
Vance C,Rogelj B,Hortobágyi T,De Vos KJ,Nishimura AL,Sreedharan J,Hu X,Smith B,Ruddy D,Wright P,Ganesalingam J,Williams KL,Tripathi V,Al-Saraj S,Al-Chalabi A,Leigh PN,Blair IP,Nicholson G,de Belleroche J,Gallo JM,Miller CC,Shaw CE
Fri Feb 27 00:00:00 EST 2009
Accumulation of phosphorylated TDP-43 in brains of patients with argyrophilic grain disease.
Acta neuropathologica
Fujishiro H,Uchikado H,Arai T,Hasegawa M,Akiyama H,Yokota O,Tsuchiya K,Togo T,Iseki E,Hirayasu Y
Sun Feb 01 00:00:00 EST 2009
Rapidly progressive neurodegenerative dementias.
Archives of neurology
Josephs KA,Ahlskog JE,Parisi JE,Boeve BF,Crum BA,Giannini C,Petersen RC
Sun Feb 01 00:00:00 EST 2009
TDP-43 accumulation is common in myopathies with rimmed vacuoles.
Acta neuropathologica
Küsters B,van Hoeve BJ,Schelhaas HJ,Ter Laak H,van Engelen BG,Lammens M
Sun Feb 01 00:00:00 EST 2009
Clinical and pathological continuum of multisystem TDP-43 proteinopathies.
Archives of neurology
Geser F,Martinez-Lage M,Robinson J,Uryu K,Neumann M,Brandmeir NJ,Xie SX,Kwong LK,Elman L,McCluskey L,Clark CM,Malunda J,Miller BL,Zimmerman EA,Qian J,Van Deerlin V,Grossman M,Lee VM,Trojanowski JQ
Sun Feb 01 00:00:00 EST 2009
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies.
Acta neuropathologica
Neumann M,Kwong LK,Lee EB,Kremmer E,Flatley A,Xu Y,Forman MS,Troost D,Kretzschmar HA,Trojanowski JQ,Lee VM
Sun Feb 01 00:00:00 EST 2009
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies.
Acta neuropathologica
Neumann M,Kwong LK,Lee EB,Kremmer E,Flatley A,Xu Y,Forman MS,Troost D,Kretzschmar HA,Trojanowski JQ,Lee VM
Sun Feb 01 00:00:00 EST 2009
Phosphorylated TDP-43 in Alzheimer's disease and dementia with Lewy bodies.
Acta neuropathologica
Arai T,Mackenzie IR,Hasegawa M,Nonoka T,Niizato K,Tsuchiya K,Iritani S,Onaya M,Akiyama H
Sun Feb 01 00:00:00 EST 2009
Phosphorylated TDP-43 in Alzheimer's disease and dementia with Lewy bodies.
Acta neuropathologica
Arai T,Mackenzie IR,Hasegawa M,Nonoka T,Niizato K,Tsuchiya K,Iritani S,Onaya M,Akiyama H
Sun Feb 01 00:00:00 EST 2009
Wobbler mice modeling motor neuron disease display elevated transactive response DNA binding protein.
Neuroscience
Dennis JS,Citron BA
Fri Jan 23 00:00:00 EST 2009
Phosphorylated and ubiquitinated TDP-43 pathological inclusions in ALS and FTLD-U are recapitulated in SH-SY5Y cells.
FEBS letters
Nonaka T,Arai T,Buratti E,Baralle FE,Akiyama H,Hasegawa M
Thu Jan 22 00:00:00 EST 2009
Phosphorylated and ubiquitinated TDP-43 pathological inclusions in ALS and FTLD-U are recapitulated in SH-SY5Y cells.
FEBS letters
Nonaka T,Arai T,Buratti E,Baralle FE,Akiyama H,Hasegawa M
Thu Jan 22 00:00:00 EST 2009
Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: implications for TDP-43 in the physiological response to neuronal injury.
Brain research
Moisse K,Volkening K,Leystra-Lantz C,Welch I,Hill T,Strong MJ
Fri Jan 16 00:00:00 EST 2009
Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: implications for TDP-43 in the physiological response to neuronal injury.
Brain research
Moisse K,Volkening K,Leystra-Lantz C,Welch I,Hill T,Strong MJ
Fri Jan 16 00:00:00 EST 2009
Clinical and pathological characteristics of patients with leucine-rich repeat kinase-2 mutations.
Movement disorders : official journal of the Movement Disorder Society
Covy JP,Yuan W,Waxman EA,Hurtig HI,Van Deerlin VM,Giasson BI
Thu Jan 15 00:00:00 EST 2009
Progression of hippocampal degeneration in amyotrophic lateral sclerosis with or without memory impairment: distinction from Alzheimer disease.
Acta neuropathologica
Takeda T,Uchihara T,Arai N,Mizutani T,Iwata M
Thu Jan 01 00:00:00 EST 2009
Nuclear TAR DNA binding protein 43 expression in spinal cord neurons correlates with the clinical course in amyotrophic lateral sclerosis.
Journal of neuropathology and experimental neurology
Sumi H,Kato S,Mochimaru Y,Fujimura H,Etoh M,Sakoda S
Thu Jan 01 00:00:00 EST 2009
Sporadic amyotrophic lateral sclerosis of long duration is associated with relatively mild TDP-43 pathology.
Acta neuropathologica
Nishihira Y,Tan CF,Hoshi Y,Iwanaga K,Yamada M,Kawachi I,Tsujihata M,Hozumi I,Morita T,Onodera O,Nishizawa M,Kakita A,Takahashi H
Thu Jan 01 00:00:00 EST 2009
Increased TDP-43 protein in cerebrospinal fluid of patients with amyotrophic lateral sclerosis.
Acta neuropathologica
Kasai T,Tokuda T,Ishigami N,Sasayama H,Foulds P,Mitchell DJ,Mann DM,Allsop D,Nakagawa M
Thu Jan 01 00:00:00 EST 2009
Increased TDP-43 protein in cerebrospinal fluid of patients with amyotrophic lateral sclerosis.
Acta neuropathologica
Kasai T,Tokuda T,Ishigami N,Sasayama H,Foulds P,Mitchell DJ,Mann DM,Allsop D,Nakagawa M
Thu Jan 01 00:00:00 EST 2009
LRRK2 and parkin immunoreactivity in multiple system atrophy inclusions.
Acta neuropathologica
Huang Y,Song YJ,Murphy K,Holton JL,Lashley T,Revesz T,Gai WP,Halliday GM
Mon Dec 01 00:00:00 EST 2008
Common variation in the miR-659 binding-site of GRN is a major risk factor for TDP43-positive frontotemporal dementia.
Human molecular genetics
Rademakers R,Eriksen JL,Baker M,Robinson T,Ahmed Z,Lincoln SJ,Finch N,Rutherford NJ,Crook RJ,Josephs KA,Boeve BF,Knopman DS,Petersen RC,Parisi JE,Caselli RJ,Wszolek ZK,Uitti RJ,Feldman H,Hutton ML,Mackenzie IR,Graff-Radford NR,Dickson DW
Mon Dec 01 00:00:00 EST 2008
Fine structural analysis of the neuronal inclusions of frontotemporal lobar degeneration with TDP-43 proteinopathy.
Journal of neural transmission (Vienna, Austria : 1996)
Thorpe JR,Tang H,Atherton J,Cairns NJ
Mon Dec 01 00:00:00 EST 2008
Colocalization of transactivation-responsive DNA-binding protein 43 and huntingtin in inclusions of Huntington disease.
Journal of neuropathology and experimental neurology
Schwab C,Arai T,Hasegawa M,Yu S,McGeer PL
Mon Dec 01 00:00:00 EST 2008
Enduring involvement of tau, beta-amyloid, alpha-synuclein, ubiquitin and TDP-43 pathology in the amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam (ALS/PDC).
Acta neuropathologica
Miklossy J,Steele JC,Yu S,McCall S,Sandberg G,McGeer EG,McGeer PL
Mon Dec 01 00:00:00 EST 2008
Early onset familial Alzheimer Disease with spastic paraparesis, dysarthria, and seizures and N135S mutation in PSEN1.
Alzheimer disease and associated disorders
Rudzinski LA,Fletcher RM,Dickson DW,Crook R,Hutton ML,Adamson J,Graff-Radford NR
Tue Nov 18 00:00:00 EST 2008
Structural determinants of the cellular localization and shuttling of TDP-43.
Journal of cell science
Ayala YM,Zago P,D'Ambrogio A,Xu YF,Petrucelli L,Buratti E,Baralle FE
Sat Nov 15 00:00:00 EST 2008
Structural determinants of the cellular localization and shuttling of TDP-43.
Journal of cell science
Ayala YM,Zago P,D'Ambrogio A,Xu YF,Petrucelli L,Buratti E,Baralle FE
Sat Nov 15 00:00:00 EST 2008
Progranulin mutation causes frontotemporal dementia in the Swedish Karolinska family.
Alzheimer's & dementia : the journal of the Alzheimer's Association
Chiang HH,Rosvall L,Brohede J,Axelman K,Björk BF,Nennesmo I,Robins T,Graff C
Sat Nov 01 00:00:00 EDT 2008
TDP-43 in cerebrospinal fluid of patients with frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Archives of neurology
Steinacker P,Hendrich C,Sperfeld AD,Jesse S,von Arnim CA,Lehnert S,Pabst A,Uttner I,Tumani H,Lee VM,Trojanowski JQ,Kretzschmar HA,Ludolph A,Neumann M,Otto M
Sat Nov 01 00:00:00 EDT 2008
TDP-43 expression in mouse models of amyotrophic lateral sclerosis and spinal muscular atrophy.
BMC neuroscience
Turner BJ,Bäumer D,Parkinson NJ,Scaber J,Ansorge O,Talbot K
Tue Oct 28 00:00:00 EDT 2008
TDP-43 expression in mouse models of amyotrophic lateral sclerosis and spinal muscular atrophy.
BMC neuroscience
Turner BJ,Bäumer D,Parkinson NJ,Scaber J,Ansorge O,Talbot K
Tue Oct 28 00:00:00 EDT 2008
Immunohistochemical identification of messenger RNA-related proteins in basophilic inclusions of adult-onset atypical motor neuron disease.
Acta neuropathologica
Fujita K,Ito H,Nakano S,Kinoshita Y,Wate R,Kusaka H
Wed Oct 01 00:00:00 EDT 2008
TDP-43 accumulation in inclusion body myopathy muscle suggests a common pathogenic mechanism with frontotemporal dementia.
Journal of neurology, neurosurgery, and psychiatry
Weihl CC,Temiz P,Miller SE,Watts G,Smith C,Forman M,Hanson PI,Kimonis V,Pestronk A
Wed Oct 01 00:00:00 EDT 2008
TDP-43 accumulation in inclusion body myopathy muscle suggests a common pathogenic mechanism with frontotemporal dementia.
Journal of neurology, neurosurgery, and psychiatry
Weihl CC,Temiz P,Miller SE,Watts G,Smith C,Forman M,Hanson PI,Kimonis V,Pestronk A
Wed Oct 01 00:00:00 EDT 2008
White matter tauopathy with globular glial inclusions: a distinct sporadic frontotemporal lobar degeneration.
Journal of neuropathology and experimental neurology
Kovacs GG,Majtenyi K,Spina S,Murrell JR,Gelpi E,Hoftberger R,Fraser G,Crowther RA,Goedert M,Budka H,Ghetti B
Wed Oct 01 00:00:00 EDT 2008
Novel mutations in TARDBP (TDP-43) in patients with familial amyotrophic lateral sclerosis.
PLoS genetics
Rutherford NJ,Zhang YJ,Baker M,Gass JM,Finch NA,Xu YF,Stewart H,Kelley BJ,Kuntz K,Crook RJ,Sreedharan J,Vance C,Sorenson E,Lippa C,Bigio EH,Geschwind DH,Knopman DS,Mitsumoto H,Petersen RC,Cashman NR,Hutton M,Shaw CE,Boylan KB,Boeve B,Graff-Radford NR,Wszolek ZK,Caselli RJ,Dickson DW,Mackenzie IR,Petrucelli L,Rademakers R
Fri Sep 19 00:00:00 EDT 2008
Caspase-cleaved TAR DNA-binding protein-43 is a major pathological finding in Alzheimer's disease.
Brain research
Rohn TT
Thu Sep 04 00:00:00 EDT 2008
Caspase-cleaved TAR DNA-binding protein-43 is a major pathological finding in Alzheimer's disease.
Brain research
Rohn TT
Thu Sep 04 00:00:00 EDT 2008
Frontotemporal dementia mimicking dementia with Lewy bodies.
Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive Neurology
Claassen DO,Parisi JE,Giannini C,Boeve BF,Dickson DW,Josephs KA
Mon Sep 01 00:00:00 EDT 2008
Pedigree with frontotemporal lobar degeneration--motor neuron disease and Tar DNA binding protein-43 positive neuropathology: genetic linkage to chromosome 9.
BMC neurology
Luty AA,Kwok JB,Thompson EM,Blumbergs P,Brooks WS,Loy CT,Dobson-Stone C,Panegyres PK,Hecker J,Nicholson GA,Halliday GM,Schofield PR
Fri Aug 29 00:00:00 EDT 2008
Abnormal phosphorylation of Ser409/410 of TDP-43 in FTLD-U and ALS.
FEBS letters
Inukai Y,Nonaka T,Arai T,Yoshida M,Hashizume Y,Beach TG,Buratti E,Baralle FE,Akiyama H,Hisanaga S,Hasegawa M
Wed Aug 20 00:00:00 EDT 2008
Abnormal phosphorylation of Ser409/410 of TDP-43 in FTLD-U and ALS.
FEBS letters
Inukai Y,Nonaka T,Arai T,Yoshida M,Hashizume Y,Beach TG,Buratti E,Baralle FE,Akiyama H,Hisanaga S,Hasegawa M
Wed Aug 20 00:00:00 EDT 2008
Ultrastructural localization of TDP-43 in filamentous neuronal inclusions in various neurodegenerative diseases.
Acta neuropathologica
Lin WL,Dickson DW
Fri Aug 01 00:00:00 EDT 2008
Temporal lobar predominance of TDP-43 neuronal cytoplasmic inclusions in Alzheimer disease.
Acta neuropathologica
Hu WT,Josephs KA,Knopman DS,Boeve BF,Dickson DW,Petersen RC,Parisi JE
Fri Aug 01 00:00:00 EDT 2008
TDP-43 protein in plasma may index TDP-43 brain pathology in Alzheimer's disease and frontotemporal lobar degeneration.
Acta neuropathologica
Foulds P,McAuley E,Gibbons L,Davidson Y,Pickering-Brown SM,Neary D,Snowden JS,Allsop D,Mann DM
Fri Aug 01 00:00:00 EDT 2008
TDP-43 protein in plasma may index TDP-43 brain pathology in Alzheimer's disease and frontotemporal lobar degeneration.
Acta neuropathologica
Foulds P,McAuley E,Gibbons L,Davidson Y,Pickering-Brown SM,Neary D,Snowden JS,Allsop D,Mann DM
Fri Aug 01 00:00:00 EDT 2008
Frontotemporal lobar degeneration with ubiquitin-positive, but TDP-43-negative inclusions.
Acta neuropathologica
Josephs KA,Lin WL,Ahmed Z,Stroh DA,Graff-Radford NR,Dickson DW
Fri Aug 01 00:00:00 EDT 2008
Maturation process of TDP-43-positive neuronal cytoplasmic inclusions in amyotrophic lateral sclerosis with and without dementia.
Acta neuropathologica
Mori F,Tanji K,Zhang HX,Nishihira Y,Tan CF,Takahashi H,Wakabayashi K
Fri Aug 01 00:00:00 EDT 2008
Lack of TAR-DNA binding protein-43 (TDP-43) pathology in human prion diseases.
Neuropathology and applied neurobiology
Isaacs AM,Powell C,Webb TE,Linehan JM,Collinge J,Brandner S
Fri Aug 01 00:00:00 EDT 2008
TDP-43-negative FTLD-U is a significant new clinico-pathological subtype of FTLD.
Acta neuropathologica
Roeber S,Mackenzie IR,Kretzschmar HA,Neumann M
Fri Aug 01 00:00:00 EDT 2008
TDP-43 inclusions do not protect motor neurons from sporadic ALS.
Acta neuropathologica
Pamphlett R,Kum Jew S
Fri Aug 01 00:00:00 EDT 2008
Sporadic amyotrophic lateral sclerosis: two pathological patterns shown by analysis of distribution of TDP-43-immunoreactive neuronal and glial cytoplasmic inclusions.
Acta neuropathologica
Nishihira Y,Tan CF,Onodera O,Toyoshima Y,Yamada M,Morita T,Nishizawa M,Kakita A,Takahashi H
Fri Aug 01 00:00:00 EDT 2008
Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Annals of neurology
Hasegawa M,Arai T,Nonaka T,Kametani F,Yoshida M,Hashizume Y,Beach TG,Buratti E,Baralle F,Morita M,Nakano I,Oda T,Tsuchiya K,Akiyama H
Tue Jul 01 00:00:00 EDT 2008
Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Annals of neurology
Hasegawa M,Arai T,Nonaka T,Kametani F,Yoshida M,Hashizume Y,Beach TG,Buratti E,Baralle F,Morita M,Nakano I,Oda T,Tsuchiya K,Akiyama H
Tue Jul 01 00:00:00 EDT 2008
Enrichment of C-terminal fragments in TAR DNA-binding protein-43 cytoplasmic inclusions in brain but not in spinal cord of frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
The American journal of pathology
Igaz LM,Kwong LK,Xu Y,Truax AC,Uryu K,Neumann M,Clark CM,Elman LB,Miller BL,Grossman M,McCluskey LF,Trojanowski JQ,Lee VM
Tue Jul 01 00:00:00 EDT 2008
Enrichment of C-terminal fragments in TAR DNA-binding protein-43 cytoplasmic inclusions in brain but not in spinal cord of frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
The American journal of pathology
Igaz LM,Kwong LK,Xu Y,Truax AC,Uryu K,Neumann M,Clark CM,Elman LB,Miller BL,Grossman M,McCluskey LF,Trojanowski JQ,Lee VM
Tue Jul 01 00:00:00 EDT 2008
Clinical, neuropathological and genotypic variability in SNCA A53T familial Parkinson's disease. Variability in familial Parkinson's disease.
Acta neuropathologica
Markopoulou K,Dickson DW,McComb RD,Wszolek ZK,Katechalidou L,Avery L,Stansbury MS,Chase BA
Tue Jul 01 00:00:00 EDT 2008
Update on recent molecular and genetic advances in frontotemporal lobar degeneration.
Journal of neuropathology and experimental neurology
Bigio EH
Tue Jul 01 00:00:00 EDT 2008
A90V TDP-43 variant results in the aberrant localization of TDP-43 in vitro.
FEBS letters
Winton MJ,Van Deerlin VM,Kwong LK,Yuan W,Wood EM,Yu CE,Schellenberg GD,Rademakers R,Caselli R,Karydas A,Trojanowski JQ,Miller BL,Lee VM
Wed Jun 25 00:00:00 EDT 2008
A90V TDP-43 variant results in the aberrant localization of TDP-43 in vitro.
FEBS letters
Winton MJ,Van Deerlin VM,Kwong LK,Yuan W,Wood EM,Yu CE,Schellenberg GD,Rademakers R,Caselli R,Karydas A,Trojanowski JQ,Miller BL,Lee VM
Wed Jun 25 00:00:00 EDT 2008
Progranulin gene mutation with an unusual clinical and neuropathologic presentation.
Movement disorders : official journal of the Movement Disorder Society
Wider C,Uitti RJ,Wszolek ZK,Fang JY,Josephs KA,Baker MC,Rademakers R,Hutton ML,Dickson DW
Sun Jun 15 00:00:00 EDT 2008
Anterior horn cells with abnormal TDP-43 immunoreactivities show fragmentation of the Golgi apparatus in ALS.
Journal of the neurological sciences
Fujita Y,Mizuno Y,Takatama M,Okamoto K
Sun Jun 15 00:00:00 EDT 2008
Concomitant TAR-DNA-binding protein 43 pathology is present in Alzheimer disease and corticobasal degeneration but not in other tauopathies.
Journal of neuropathology and experimental neurology
Uryu K,Nakashima-Yasuda H,Forman MS,Kwong LK,Clark CM,Grossman M,Miller BL,Kretzschmar HA,Lee VM,Trojanowski JQ,Neumann M
Sun Jun 01 00:00:00 EDT 2008
Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia.
Annals of neurology
Mesulam M,Wicklund A,Johnson N,Rogalski E,Léger GC,Rademaker A,Weintraub S,Bigio EH
Sun Jun 01 00:00:00 EDT 2008
Disturbance of nuclear and cytoplasmic TAR DNA-binding protein (TDP-43) induces disease-like redistribution, sequestration, and aggregate formation.
The Journal of biological chemistry
Winton MJ,Igaz LM,Wong MM,Kwong LK,Trojanowski JQ,Lee VM
Fri May 09 00:00:00 EDT 2008
Disturbance of nuclear and cytoplasmic TAR DNA-binding protein (TDP-43) induces disease-like redistribution, sequestration, and aggregate formation.
The Journal of biological chemistry
Winton MJ,Igaz LM,Wong MM,Kwong LK,Trojanowski JQ,Lee VM
Fri May 09 00:00:00 EDT 2008
Disturbance of nuclear and cytoplasmic TAR DNA-binding protein (TDP-43) induces disease-like redistribution, sequestration, and aggregate formation.
The Journal of biological chemistry
Winton MJ,Igaz LM,Wong MM,Kwong LK,Trojanowski JQ,Lee VM
Fri May 09 00:00:00 EDT 2008
Abnormal TDP-43 immunoreactivity in AD modifies clinicopathologic and radiologic phenotype.
Neurology
Josephs KA,Whitwell JL,Knopman DS,Hu WT,Stroh DA,Baker M,Rademakers R,Boeve BF,Parisi JE,Smith GE,Ivnik RJ,Petersen RC,Jack CR,Dickson DW
Tue May 06 00:00:00 EDT 2008
Pilot study of granulocyte colony stimulating factor (G-CSF)-mobilized peripheral blood stem cells in amyotrophic lateral sclerosis (ALS).
Muscle & nerve
Cashman N,Tan LY,Krieger C,Mädler B,Mackay A,Mackenzie I,Benny B,Nantel S,Fabros M,Shinobu L,Yousefi M,Eisen A
Thu May 01 00:00:00 EDT 2008
Atypical frontotemporal lobar degeneration with ubiquitin-positive, TDP-43-negative neuronal inclusions.
Brain : a journal of neurology
Mackenzie IR,Foti D,Woulfe J,Hurwitz TA
Thu May 01 00:00:00 EDT 2008
Evidence of multisystem disorder in whole-brain map of pathological TDP-43 in amyotrophic lateral sclerosis.
Archives of neurology
Geser F,Brandmeir NJ,Kwong LK,Martinez-Lage M,Elman L,McCluskey L,Xie SX,Lee VM,Trojanowski JQ
Thu May 01 00:00:00 EDT 2008
Basophilic inclusion body disease and neuronal intermediate filament inclusion disease: a comparative clinicopathological study.
Acta neuropathologica
Yokota O,Tsuchiya K,Terada S,Ishizu H,Uchikado H,Ikeda M,Oyanagi K,Nakano I,Murayama S,Kuroda S,Akiyama H
Thu May 01 00:00:00 EDT 2008
Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions visualized with ubiquitin-binding protein p62 immunohistochemistry.
Journal of neuropathology and experimental neurology
Pikkarainen M,Hartikainen P,Alafuzoff I
Tue Apr 01 00:00:00 EDT 2008
TAR DNA-binding protein 43 immunohistochemistry reveals extensive neuritic pathology in FTLD-U: a midwest-southwest consortium for FTLD study.
Journal of neuropathology and experimental neurology
Hatanpaa KJ,Bigio EH,Cairns NJ,Womack KB,Weintraub S,Morris JC,Foong C,Xiao G,Hladik C,Mantanona TY,White CL
Tue Apr 01 00:00:00 EDT 2008
TDP-43 mutation in familial amyotrophic lateral sclerosis.
Annals of neurology
Yokoseki A,Shiga A,Tan CF,Tagawa A,Kaneko H,Koyama A,Eguchi H,Tsujino A,Ikeuchi T,Kakita A,Okamoto K,Nishizawa M,Takahashi H,Onodera O
Tue Apr 01 00:00:00 EDT 2008
TDP-43 mutation in familial amyotrophic lateral sclerosis.
Annals of neurology
Yokoseki A,Shiga A,Tan CF,Tagawa A,Kaneko H,Koyama A,Eguchi H,Tsujino A,Ikeuchi T,Kakita A,Okamoto K,Nishizawa M,Takahashi H,Onodera O
Tue Apr 01 00:00:00 EDT 2008
Epitope mapping of 2E2-D3, a monoclonal antibody directed against human TDP-43.
Neuroscience letters
Zhang HX,Tanji K,Mori F,Wakabayashi K
Fri Mar 28 00:00:00 EDT 2008
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis.
Science (New York, N.Y.)
Sreedharan J,Blair IP,Tripathi VB,Hu X,Vance C,Rogelj B,Ackerley S,Durnall JC,Williams KL,Buratti E,Baralle F,de Belleroche J,Mitchell JD,Leigh PN,Al-Chalabi A,Miller CC,Nicholson G,Shaw CE
Fri Mar 21 00:00:00 EDT 2008
TDP-43 immunoreactivity in anoxic, ischemic and neoplastic lesions of the central nervous system.
Acta neuropathologica
Lee EB,Lee VM,Trojanowski JQ,Neumann M
Sat Mar 01 00:00:00 EST 2008
Frequency and clinical characteristics of progranulin mutation carriers in the Manchester frontotemporal lobar degeneration cohort: comparison with patients with MAPT and no known mutations.
Brain : a journal of neurology
Pickering-Brown SM,Rollinson S,Du Plessis D,Morrison KE,Varma A,Richardson AM,Neary D,Snowden JS,Mann DM
Sat Mar 01 00:00:00 EST 2008
Phosphorylated Smad2/3 immunoreactivity in sporadic and familial amyotrophic lateral sclerosis and its mouse model.
Acta neuropathologica
Nakamura M,Ito H,Wate R,Nakano S,Hirano A,Kusaka H
Sat Mar 01 00:00:00 EST 2008
Liver X receptor beta (LXRbeta): a link between beta-sitosterol and amyotrophic lateral sclerosis-Parkinson's dementia.
Proceedings of the National Academy of Sciences of the United States of America
Kim HJ,Fan X,Gabbi C,Yakimchuk K,Parini P,Warner M,Gustafsson JA
Tue Feb 12 00:00:00 EST 2008
Missense mutations in the progranulin gene linked to frontotemporal lobar degeneration with ubiquitin-immunoreactive inclusions reduce progranulin production and secretion.
The Journal of biological chemistry
Shankaran SS,Capell A,Hruscha AT,Fellerer K,Neumann M,Schmid B,Haass C
Fri Jan 18 00:00:00 EST 2008
Missense mutations in the progranulin gene linked to frontotemporal lobar degeneration with ubiquitin-immunoreactive inclusions reduce progranulin production and secretion.
The Journal of biological chemistry
Shankaran SS,Capell A,Hruscha AT,Fellerer K,Neumann M,Schmid B,Haass C
Fri Jan 18 00:00:00 EST 2008
Missense mutations in the progranulin gene linked to frontotemporal lobar degeneration with ubiquitin-immunoreactive inclusions reduce progranulin production and secretion.
The Journal of biological chemistry
Shankaran SS,Capell A,Hruscha AT,Fellerer K,Neumann M,Schmid B,Haass C
Fri Jan 18 00:00:00 EST 2008
TAR-DNA binding protein 43 in Pick disease.
Journal of neuropathology and experimental neurology
Freeman SH,Spires-Jones T,Hyman BT,Growdon JH,Frosch MP
Tue Jan 01 00:00:00 EST 2008
Pathological TDP-43 in parkinsonism-dementia complex and amyotrophic lateral sclerosis of Guam.
Acta neuropathologica
Geser F,Winton MJ,Kwong LK,Xu Y,Xie SX,Igaz LM,Garruto RM,Perl DP,Galasko D,Lee VM,Trojanowski JQ
Tue Jan 01 00:00:00 EST 2008
Pathological TDP-43 in parkinsonism-dementia complex and amyotrophic lateral sclerosis of Guam.
Acta neuropathologica
Geser F,Winton MJ,Kwong LK,Xu Y,Xie SX,Igaz LM,Garruto RM,Perl DP,Galasko D,Lee VM,Trojanowski JQ
Tue Jan 01 00:00:00 EST 2008
Pathological TDP-43 in parkinsonism-dementia complex and amyotrophic lateral sclerosis of Guam.
Acta neuropathologica
Geser F,Winton MJ,Kwong LK,Xu Y,Xie SX,Igaz LM,Garruto RM,Perl DP,Galasko D,Lee VM,Trojanowski JQ
Tue Jan 01 00:00:00 EST 2008
Severe subcortical TDP-43 pathology in sporadic frontotemporal lobar degeneration with motor neuron disease.
Acta neuropathologica
Brandmeir NJ,Geser F,Kwong LK,Zimmerman E,Qian J,Lee VM,Trojanowski JQ
Tue Jan 01 00:00:00 EST 2008
TDP-43-immunoreactive neuronal and glial inclusions in the neostriatum in amyotrophic lateral sclerosis with and without dementia.
Acta neuropathologica
Zhang H,Tan CF,Mori F,Tanji K,Kakita A,Takahashi H,Wakabayashi K
Tue Jan 01 00:00:00 EST 2008
Concurrence of TDP-43, tau and alpha-synuclein pathology in brains of Alzheimer's disease and dementia with Lewy bodies.
Brain research
Higashi S,Iseki E,Yamamoto R,Minegishi M,Hino H,Fujisawa K,Togo T,Katsuse O,Uchikado H,Furukawa Y,Kosaka K,Arai H
Wed Dec 12 00:00:00 EST 2007
Evidence that TDP-43 is not the major ubiquitinated target within the pathological inclusions of amyotrophic lateral sclerosis.
Journal of neuropathology and experimental neurology
Sanelli T,Xiao S,Horne P,Bilbao J,Zinman L,Robertson J
Sat Dec 01 00:00:00 EST 2007
Functional multivesicular bodies are required for autophagic clearance of protein aggregates associated with neurodegenerative disease.
The Journal of cell biology
Filimonenko M,Stuffers S,Raiborg C,Yamamoto A,Malerød L,Fisher EM,Isaacs A,Brech A,Stenmark H,Simonsen A
Mon Nov 05 00:00:00 EST 2007
Functional multivesicular bodies are required for autophagic clearance of protein aggregates associated with neurodegenerative disease.
The Journal of cell biology
Filimonenko M,Stuffers S,Raiborg C,Yamamoto A,Malerød L,Fisher EM,Isaacs A,Brech A,Stenmark H,Simonsen A
Mon Nov 05 00:00:00 EST 2007
Increase in the relative expression of tau with four microtubule binding repeat regions in frontotemporal lobar degeneration and progressive supranuclear palsy brains.
Acta neuropathologica
Ingelsson M,Ramasamy K,Russ C,Freeman SH,Orne J,Raju S,Matsui T,Growdon JH,Frosch MP,Ghetti B,Brown RH,Irizarry MC,Hyman BT
Thu Nov 01 00:00:00 EDT 2007
Frontotemporal lobar degeneration with upper motor neuron disease/ primary lateral sclerosis.
Neurology
Josephs KA,Dickson DW
Tue Oct 30 00:00:00 EDT 2007
A reassessment of the neuropathology of frontotemporal dementia linked to chromosome 3.
Journal of neuropathology and experimental neurology
Holm IE,Englund E,Mackenzie IR,Johannsen P,Isaacs AM
Mon Oct 01 00:00:00 EDT 2007
Alzheimer and Parkinson diagnoses in progranulin null mutation carriers in an extended founder family.
Archives of neurology
Brouwers N,Nuytemans K,van der Zee J,Gijselinck I,Engelborghs S,Theuns J,Kumar-Singh S,Pickut BA,Pals P,Dermaut B,Bogaerts V,De Pooter T,Serneels S,Van den Broeck M,Cuijt I,Mattheijssens M,Peeters K,Sciot R,Martin JJ,Cras P,Santens P,Vandenberghe R,De Deyn PP,Cruts M,Van Broeckhoven C,Sleegers K
Mon Oct 01 00:00:00 EDT 2007
Progressive anomia revisited: focal degeneration associated with progranulin gene mutation.
Neurocase
Snowden JS,Pickering-Brown SM,Du Plessis D,Mackenzie IR,Varma A,Mann DM,Neary D
Mon Oct 01 00:00:00 EDT 2007
Corticobasal syndrome associated with the A9D Progranulin mutation.
Journal of neuropathology and experimental neurology
Spina S,Murrell JR,Huey ED,Wassermann EM,Pietrini P,Grafman J,Ghetti B
Mon Oct 01 00:00:00 EDT 2007
Progranulin mediates caspase-dependent cleavage of TAR DNA binding protein-43.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Zhang YJ,Xu YF,Dickey CA,Buratti E,Baralle F,Bailey R,Pickering-Brown S,Dickson D,Petrucelli L
Wed Sep 26 00:00:00 EDT 2007
Progranulin mediates caspase-dependent cleavage of TAR DNA binding protein-43.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Zhang YJ,Xu YF,Dickey CA,Buratti E,Baralle F,Bailey R,Pickering-Brown S,Dickson D,Petrucelli L
Wed Sep 26 00:00:00 EDT 2007
Co-morbidity of TDP-43 proteinopathy in Lewy body related diseases.
Acta neuropathologica
Nakashima-Yasuda H,Uryu K,Robinson J,Xie SX,Hurtig H,Duda JE,Arnold SE,Siderowf A,Grossman M,Leverenz JB,Woltjer R,Lopez OL,Hamilton R,Tsuang DW,Galasko D,Masliah E,Kaye J,Clark CM,Montine TJ,Lee VM,Trojanowski JQ
Sat Sep 01 00:00:00 EDT 2007
TDP-43 in differential diagnosis of motor neuron disorders.
Acta neuropathologica
Dickson DW,Josephs KA,Amador-Ortiz C
Sun Jul 01 00:00:00 EDT 2007
TDP-43 in familial and sporadic frontotemporal lobar degeneration with ubiquitin inclusions.
The American journal of pathology
Cairns NJ,Neumann M,Bigio EH,Holm IE,Troost D,Hatanpaa KJ,Foong C,White CL,Schneider JA,Kretzschmar HA,Carter D,Taylor-Reinwald L,Paulsmeyer K,Strider J,Gitcho M,Goate AM,Morris JC,Mishra M,Kwong LK,Stieber A,Xu Y,Forman MS,Trojanowski JQ,Lee VM,Mackenzie IR
Sun Jul 01 00:00:00 EDT 2007
Lack of TDP-43 abnormalities in mutant SOD1 transgenic mice shows disparity with ALS.
Neuroscience letters
Robertson J,Sanelli T,Xiao S,Yang W,Horne P,Hammond R,Pioro EP,Strong MJ
Wed Jun 13 00:00:00 EDT 2007
Lack of TDP-43 abnormalities in mutant SOD1 transgenic mice shows disparity with ALS.
Neuroscience letters
Robertson J,Sanelli T,Xiao S,Yang W,Horne P,Hammond R,Pioro EP,Strong MJ
Wed Jun 13 00:00:00 EDT 2007
Appearance pattern of TDP-43 in Japanese frontotemporal lobar degeneration with ubiquitin-positive inclusions.
Neuroscience letters
Higashi S,Iseki E,Yamamoto R,Minegishi M,Hino H,Fujisawa K,Togo T,Katsuse O,Uchikado H,Furukawa Y,Kosaka K,Arai H
Mon Jun 04 00:00:00 EDT 2007
Nuclear protein profiling of Jurkat cells during heat stress-induced apoptosis by 2-DE and MS/MS.
Electrophoresis
Yuan X,Kuramitsu Y,Furumoto H,Zhang X,Hayashi E,Fujimoto M,Nakamura K
Fri Jun 01 00:00:00 EDT 2007
Ubiquitinated pathological lesions in frontotemporal lobar degeneration contain the TAR DNA-binding protein, TDP-43.
Acta neuropathologica
Davidson Y,Kelley T,Mackenzie IR,Pickering-Brown S,Du Plessis D,Neary D,Snowden JS,Mann DM
Tue May 01 00:00:00 EDT 2007
TDP-43 is deposited in the Guam parkinsonism-dementia complex brains.
Brain : a journal of neurology
Hasegawa M,Arai T,Akiyama H,Nonaka T,Mori H,Hashimoto T,Yamazaki M,Oyanagi K
Tue May 01 00:00:00 EDT 2007
TDP-43 is deposited in the Guam parkinsonism-dementia complex brains.
Brain : a journal of neurology
Hasegawa M,Arai T,Akiyama H,Nonaka T,Mori H,Hashimoto T,Yamazaki M,Oyanagi K
Tue May 01 00:00:00 EDT 2007
Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations.
Annals of neurology
Mackenzie IR,Bigio EH,Ince PG,Geser F,Neumann M,Cairns NJ,Kwong LK,Forman MS,Ravits J,Stewart H,Eisen A,McClusky L,Kretzschmar HA,Monoranu CM,Highley JR,Kirby J,Siddique T,Shaw PJ,Lee VM,Trojanowski JQ
Tue May 01 00:00:00 EDT 2007
Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations.
Annals of neurology
Mackenzie IR,Bigio EH,Ince PG,Geser F,Neumann M,Cairns NJ,Kwong LK,Forman MS,Ravits J,Stewart H,Eisen A,McClusky L,Kretzschmar HA,Monoranu CM,Highley JR,Kirby J,Siddique T,Shaw PJ,Lee VM,Trojanowski JQ
Tue May 01 00:00:00 EDT 2007
TDP-43 immunoreactivity in hippocampal sclerosis and Alzheimer's disease.
Annals of neurology
Amador-Ortiz C,Lin WL,Ahmed Z,Personett D,Davies P,Duara R,Graff-Radford NR,Hutton ML,Dickson DW
Tue May 01 00:00:00 EDT 2007
TDP-43 immunoreactivity in hippocampal sclerosis and Alzheimer's disease.
Annals of neurology
Amador-Ortiz C,Lin WL,Ahmed Z,Personett D,Davies P,Duara R,Graff-Radford NR,Hutton ML,Dickson DW
Tue May 01 00:00:00 EDT 2007
TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation.
Acta neuropathologica
Tan CF,Eguchi H,Tagawa A,Onodera O,Iwasaki T,Tsujino A,Nishizawa M,Kakita A,Takahashi H
Tue May 01 00:00:00 EDT 2007
A novel progranulin mutation associated with variable clinical presentation and tau, TDP43 and alpha-synuclein pathology.
Brain : a journal of neurology
Leverenz JB,Yu CE,Montine TJ,Steinbart E,Bekris LM,Zabetian C,Kwong LK,Lee VM,Schellenberg GD,Bird TD
Tue May 01 00:00:00 EDT 2007
A novel progranulin mutation associated with variable clinical presentation and tau, TDP43 and alpha-synuclein pathology.
Brain : a journal of neurology
Leverenz JB,Yu CE,Montine TJ,Steinbart E,Bekris LM,Zabetian C,Kwong LK,Lee VM,Schellenberg GD,Bird TD
Tue May 01 00:00:00 EDT 2007
Absence of heterogeneous nuclear ribonucleoproteins and survival motor neuron protein in TDP-43 positive inclusions in frontotemporal lobar degeneration.
Acta neuropathologica
Neumann M,Igaz LM,Kwong LK,Nakashima-Yasuda H,Kolb SJ,Dreyfuss G,Kretzschmar HA,Trojanowski JQ,Lee VM
Tue May 01 00:00:00 EDT 2007
Absence of heterogeneous nuclear ribonucleoproteins and survival motor neuron protein in TDP-43 positive inclusions in frontotemporal lobar degeneration.
Acta neuropathologica
Neumann M,Igaz LM,Kwong LK,Nakashima-Yasuda H,Kolb SJ,Dreyfuss G,Kretzschmar HA,Trojanowski JQ,Lee VM
Tue May 01 00:00:00 EDT 2007
Clinicopathologic features of frontotemporal dementia with progranulin sequence variation.
Neurology
Spina S,Murrell JR,Huey ED,Wassermann EM,Pietrini P,Baraibar MA,Barbeito AG,Troncoso JC,Vidal R,Ghetti B,Grafman J
Tue Mar 13 00:00:00 EDT 2007
TDP-43-positive white matter pathology in frontotemporal lobar degeneration with ubiquitin-positive inclusions.
Journal of neuropathology and experimental neurology
Neumann M,Kwong LK,Truax AC,Vanmassenhove B,Kretzschmar HA,Van Deerlin VM,Clark CM,Grossman M,Miller BL,Trojanowski JQ,Lee VM
Thu Mar 01 00:00:00 EST 2007
TDP-43-positive white matter pathology in frontotemporal lobar degeneration with ubiquitin-positive inclusions.
Journal of neuropathology and experimental neurology
Neumann M,Kwong LK,Truax AC,Vanmassenhove B,Kretzschmar HA,Van Deerlin VM,Clark CM,Grossman M,Miller BL,Trojanowski JQ,Lee VM
Thu Mar 01 00:00:00 EST 2007
Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with progranulin gene (PGRN) mutations.
Journal of neuropathology and experimental neurology
Josephs KA,Ahmed Z,Katsuse O,Parisi JF,Boeve BF,Knopman DS,Petersen RC,Davies P,Duara R,Graff-Radford NR,Uitti RJ,Rademakers R,Adamson J,Baker M,Hutton ML,Dickson DW
Thu Feb 01 00:00:00 EST 2007
TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations.
Journal of neuropathology and experimental neurology
Neumann M,Mackenzie IR,Cairns NJ,Boyer PJ,Markesbery WR,Smith CD,Taylor JP,Kretzschmar HA,Kimonis VE,Forman MS
Thu Feb 01 00:00:00 EST 2007
TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations.
Journal of neuropathology and experimental neurology
Neumann M,Mackenzie IR,Cairns NJ,Boyer PJ,Markesbery WR,Smith CD,Taylor JP,Kretzschmar HA,Kimonis VE,Forman MS
Thu Feb 01 00:00:00 EST 2007
A 34-year-old man with progressive behavioral and language disturbance.
Neurology
Miller BL,Josephs KA,Parisi JE,Keegan BM
Tue Jan 02 00:00:00 EST 2007
TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Biochemical and biophysical research communications
Arai T,Hasegawa M,Akiyama H,Ikeda K,Nonaka T,Mori H,Mann D,Tsuchiya K,Yoshida M,Hashizume Y,Oda T
Fri Dec 22 00:00:00 EST 2006
TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Biochemical and biophysical research communications
Arai T,Hasegawa M,Akiyama H,Ikeda K,Nonaka T,Mori H,Mann D,Tsuchiya K,Yoshida M,Hashizume Y,Oda T
Fri Dec 22 00:00:00 EST 2006
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Science (New York, N.Y.)
Neumann M,Sampathu DM,Kwong LK,Truax AC,Micsenyi MC,Chou TT,Bruce J,Schuck T,Grossman M,Clark CM,McCluskey LF,Miller BL,Masliah E,Mackenzie IR,Feldman H,Feiden W,Kretzschmar HA,Trojanowski JQ,Lee VM
Fri Oct 06 00:00:00 EDT 2006
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Science (New York, N.Y.)
Neumann M,Sampathu DM,Kwong LK,Truax AC,Micsenyi MC,Chou TT,Bruce J,Schuck T,Grossman M,Clark CM,McCluskey LF,Miller BL,Masliah E,Mackenzie IR,Feldman H,Feiden W,Kretzschmar HA,Trojanowski JQ,Lee VM
Fri Oct 06 00:00:00 EDT 2006
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Science (New York, N.Y.)
Neumann M,Sampathu DM,Kwong LK,Truax AC,Micsenyi MC,Chou TT,Bruce J,Schuck T,Grossman M,Clark CM,McCluskey LF,Miller BL,Masliah E,Mackenzie IR,Feldman H,Feiden W,Kretzschmar HA,Trojanowski JQ,Lee VM
Fri Oct 06 00:00:00 EDT 2006