Product References
ALS/FTD-Linked Mutation in FUS Suppresses Intra-axonal Protein Synthesis and Drives Disease Without Nuclear Loss-of-Function of FUS.
Neuron
López-Erauskin J,Tadokoro T,Baughn MW,Myers B,McAlonis-Downes M,Chillon-Marinas C,Asiaban JN,Artates J,Bui AT,Vetto AP,Lee SK,Le AV,Sun Y,Jambeau M,Boubaker J,Swing D,Qiu J,Hicks GG,Ouyang Z,Fu XD,Tessarollo L,Ling SC,Parone PA,Shaw CE,Marsala M,Lagier-Tourenne C,Cleveland DW,Da Cruz S
Wed Nov 21 00:00:00 EST 2018
Two herpesviral noncoding PAN RNAs are functionally homologous but do not associate with common chromatin loci.
PLoS pathogens
Withers JB,Li ES,Vallery TK,Yario TA,Steitz JA
Thu Nov 01 00:00:00 EDT 2018
Mutant FUS causes DNA ligation defects to inhibit oxidative damage repair in Amyotrophic Lateral Sclerosis.
Nature communications
Wang H,Guo W,Mitra J,Hegde PM,Vandoorne T,Eckelmann BJ,Mitra S,Tomkinson AE,Van Den Bosch L,Hegde ML
Tue Sep 11 00:00:00 EDT 2018
Elevated FUS/TLS expression is negatively associated with E-cadherin expression and prognosis of patients with non-small cell lung cancer.
Oncology letters
Xiong D,Wu YB,Jin C,Li JJ,Gu J,Liao YF,Long X,Zhu SQ,Wu HB,Xu JJ,Ding JY
Wed Aug 01 00:00:00 EDT 2018
Elevated FUS/TLS expression is negatively associated with E-cadherin expression and prognosis of patients with non-small cell lung cancer.
Oncology letters
Xiong D,Wu YB,Jin C,Li JJ,Gu J,Liao YF,Long X,Zhu SQ,Wu HB,Xu JJ,Ding JY
Wed Aug 01 00:00:00 EDT 2018
ALS-Related Mutant FUS Protein Is Mislocalized to Cytoplasm and Is Recruited into Stress Granules of Fibroblasts from Asymptomatic FUS P525L Mutation Carriers.
Neuro-degenerative diseases
Lo Bello M,Di Fini F,Notaro A,Spataro R,Conforti FL,La Bella V
Tue Jul 24 00:00:00 EDT 2018
ALS-Related Mutant FUS Protein Is Mislocalized to Cytoplasm and Is Recruited into Stress Granules of Fibroblasts from Asymptomatic FUS P525L Mutation Carriers.
Neuro-degenerative diseases
Lo Bello M,Di Fini F,Notaro A,Spataro R,Conforti FL,La Bella V
Tue Jul 24 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
Functional domains of the FSHD-associated DUX4 protein.
Biology open
Mitsuhashi H,Ishimaru S,Homma S,Yu B,Honma Y,Beermann ML,Miller JB
Thu Apr 26 00:00:00 EDT 2018
Nuclear-Import Receptors Reverse Aberrant Phase Transitions of RNA-Binding Proteins with Prion-like Domains.
Cell
Guo L,Kim HJ,Wang H,Monaghan J,Freyermuth F,Sung JC,O'Donovan K,Fare CM,Diaz Z,Singh N,Zhang ZC,Coughlin M,Sweeny EA,DeSantis ME,Jackrel ME,Rodell CB,Burdick JA,King OD,Gitler AD,Lagier-Tourenne C,Pandey UB,Chook YM,Taylor JP,Shorter J
Thu Apr 19 00:00:00 EDT 2018
Nuclear-Import Receptors Reverse Aberrant Phase Transitions of RNA-Binding Proteins with Prion-like Domains.
Cell
Guo L,Kim HJ,Wang H,Monaghan J,Freyermuth F,Sung JC,O'Donovan K,Fare CM,Diaz Z,Singh N,Zhang ZC,Coughlin M,Sweeny EA,DeSantis ME,Jackrel ME,Rodell CB,Burdick JA,King OD,Gitler AD,Lagier-Tourenne C,Pandey UB,Chook YM,Taylor JP,Shorter J
Thu Apr 19 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
Tauopathy with hippocampal 4-repeat tau immunoreactive spherical inclusions: a report of three cases.
Brain pathology (Zurich, Switzerland)
Kovacs GG,Kwong LK,Grossman M,Irwin DJ,Lee EB,Robinson JL,Suh E,Van Deerlin VM,Lee VM,Trojanowski JQ
Thu Mar 01 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
Asymmetry of post-mortem neuropathology in behavioural-variant frontotemporal dementia.
Brain : a journal of neurology
Irwin DJ,McMillan CT,Xie SX,Rascovsky K,Van Deerlin VM,Coslett HB,Hamilton R,Aguirre GK,Lee EB,Lee VMY,Trojanowski JQ,Grossman M
Mon Jan 01 00:00:00 EST 2018
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
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
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
Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS-associated amyotrophic lateral sclerosis.
Acta neuropathologica
Scekic-Zahirovic J,Oussini HE,Mersmann S,Drenner K,Wagner M,Sun Y,Allmeroth K,Dieterlé S,Sinniger J,Dirrig-Grosch S,René F,Dormann D,Haass C,Ludolph AC,Lagier-Tourenne C,Storkebaum E,Dupuis L
Thu Jun 01 00:00:00 EDT 2017
Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS-associated amyotrophic lateral sclerosis.
Acta neuropathologica
Scekic-Zahirovic J,Oussini HE,Mersmann S,Drenner K,Wagner M,Sun Y,Allmeroth K,Dieterlé S,Sinniger J,Dirrig-Grosch S,René F,Dormann D,Haass C,Ludolph AC,Lagier-Tourenne C,Storkebaum E,Dupuis L
Thu Jun 01 00:00:00 EDT 2017
Chronically stressed or stress-preconditioned neurons fail to maintain stress granule assembly.
Cell death & disease
Shelkovnikova TA,Dimasi P,Kukharsky MS,An H,Quintiero A,Schirmer C,Buée L,Galas MC,Buchman VL
Thu May 11 00:00:00 EDT 2017
FUS inclusions disrupt RNA localization by sequestering kinesin-1 and inhibiting microtubule detyrosination.
The Journal of cell biology
Yasuda K,Clatterbuck-Soper SF,Jackrel ME,Shorter J,Mili S
Mon Apr 03 00:00:00 EDT 2017
FUS inclusions disrupt RNA localization by sequestering kinesin-1 and inhibiting microtubule detyrosination.
The Journal of cell biology
Yasuda K,Clatterbuck-Soper SF,Jackrel ME,Shorter J,Mili S
Mon Apr 03 00:00:00 EDT 2017
Molecular determinants of cytochrome C oxidase IV mRNA axonal trafficking.
Molecular and cellular neurosciences
Kar AN,Vargas JNS,Chen CY,Kowalak JA,Gioio AE,Kaplan BB
Sat Apr 01 00:00:00 EDT 2017
Molecular determinants of cytochrome C oxidase IV mRNA axonal trafficking.
Molecular and cellular neurosciences
Kar AN,Vargas JNS,Chen CY,Kowalak JA,Gioio AE,Kaplan BB
Sat Apr 01 00:00:00 EDT 2017
PINK1 and Parkin are genetic modifiers for FUS-induced neurodegeneration.
Human molecular genetics
Chen Y,Deng J,Wang P,Yang M,Chen X,Zhu L,Liu J,Lu B,Shen Y,Fushimi K,Xu Q,Wu JY
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
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
Early lethality and neuronal proteinopathy in mice expressing cytoplasm-targeted FUS that lacks the RNA recognition motif.
Amyotrophic lateral sclerosis & frontotemporal degeneration
Robinson HK,Deykin AV,Bronovitsky EV,Ovchinnikov RK,Ustyugov AA,Shelkovnikova TA,Kukharsky MS,Ermolkevich TG,Goldman IL,Sadchikova ER,Kovrazhkina EA,Bachurin SO,Buchman VL,Ninkina NN
Mon Aug 01 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
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
Super-Resolution Microscopy Reveals Presynaptic Localization of the ALS/FTD Related Protein FUS in Hippocampal Neurons.
Frontiers in cellular neuroscience
Schoen M,Reichel JM,Demestre M,Putz S,Deshpande D,Proepper C,Liebau S,Schmeisser MJ,Ludolph AC,Michaelis J,Boeckers TM
Tue Feb 02 00:00:00 EST 2016
Directly converted patient-specific induced neurons mirror the neuropathology of FUS with disrupted nuclear localization in amyotrophic lateral sclerosis.
Molecular neurodegeneration
Lim SM,Choi WJ,Oh KW,Xue Y,Choi JY,Kim SH,Nahm M,Kim YE,Lee J,Noh MY,Lee S,Hwang S,Ki CS,Fu XD,Kim SH
Fri Jan 22 00:00:00 EST 2016
A new design of a lentiviral shRNA vector with inducible co-expression of ARGONAUTE 2 for enhancing gene silencing efficiency.
Cell & bioscience
He J,Huang L,Qiu H,Li J,Luo L,Li Y,Tian S,Kang K,Luo J,Liu L,Gou D
Wed Dec 09 00:00:00 EST 2015
RBM45 Modulates the Antioxidant Response in Amyotrophic Lateral Sclerosis through Interactions with KEAP1.
Molecular and cellular biology
Bakkar N,Kousari A,Kovalik T,Li Y,Bowser R
Wed Jul 01 00:00:00 EDT 2015
Mutant human FUS Is ubiquitously mislocalized and generates persistent stress granules in primary cultured transgenic zebrafish cells.
PloS one
Acosta JR,Goldsbury C,Winnick C,Badrock AP,Fraser ST,Laird AS,Hall TE,Don EK,Fifita JA,Blair IP,Nicholson GA,Cole NJ
Mon Jun 15 00:00:00 EDT 2015
Mutant human FUS Is ubiquitously mislocalized and generates persistent stress granules in primary cultured transgenic zebrafish cells.
PloS one
Acosta JR,Goldsbury C,Winnick C,Badrock AP,Fraser ST,Laird AS,Hall TE,Don EK,Fifita JA,Blair IP,Nicholson GA,Cole NJ
Mon Jun 15 00:00:00 EDT 2015
Cytoplasmic sequestration of FUS/TLS associated with ALS alters histone marks through loss of nuclear protein arginine methyltransferase 1.
Human molecular genetics
Tibshirani M,Tradewell ML,Mattina KR,Minotti S,Yang W,Zhou H,Strong MJ,Hayward LJ,Durham HD
Sun Feb 01 00:00:00 EST 2015
Cytoplasmic sequestration of FUS/TLS associated with ALS alters histone marks through loss of nuclear protein arginine methyltransferase 1.
Human molecular genetics
Tibshirani M,Tradewell ML,Mattina KR,Minotti S,Yang W,Zhou H,Strong MJ,Hayward LJ,Durham HD
Sun Feb 01 00:00:00 EST 2015
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
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
PARP-1 dependent recruitment of the amyotrophic lateral sclerosis-associated protein FUS/TLS to sites of oxidative DNA damage.
Nucleic acids research
Rulten SL,Rotheray A,Green RL,Grundy GJ,Moore DA,Gómez-Herreros F,Hafezparast M,Caldecott KW
Wed Jan 01 00:00:00 EST 2014
PARP-1 dependent recruitment of the amyotrophic lateral sclerosis-associated protein FUS/TLS to sites of oxidative DNA damage.
Nucleic acids research
Rulten SL,Rotheray A,Green RL,Grundy GJ,Moore DA,Gómez-Herreros F,Hafezparast M,Caldecott KW
Wed Jan 01 00:00:00 EST 2014
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
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
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
Mutant FUS induces endoplasmic reticulum stress in amyotrophic lateral sclerosis and interacts with protein disulfide-isomerase.
Neurobiology of aging
Farg MA,Soo KY,Walker AK,Pham H,Orian J,Horne MK,Warraich ST,Williams KL,Blair IP,Atkin JD
Sat Dec 01 00:00:00 EST 2012
Mutant FUS induces endoplasmic reticulum stress in amyotrophic lateral sclerosis and interacts with protein disulfide-isomerase.
Neurobiology of aging
Farg MA,Soo KY,Walker AK,Pham H,Orian J,Horne MK,Warraich ST,Williams KL,Blair IP,Atkin JD
Sat Dec 01 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
p62/sequestosome 1 binds to TDP-43 in brains with frontotemporal lobar degeneration with TDP-43 inclusions.
Journal of neuroscience research
Tanji K,Zhang HX,Mori F,Kakita A,Takahashi H,Wakabayashi K
Mon Oct 01 00:00:00 EDT 2012
Localization of fused in sarcoma (FUS) protein to the post-synaptic density in the brain.
Acta neuropathologica
Aoki N,Higashi S,Kawakami I,Kobayashi Z,Hosokawa M,Katsuse O,Togo T,Hirayasu Y,Akiyama H
Sat Sep 01 00:00:00 EDT 2012
Localization of fused in sarcoma (FUS) protein to the post-synaptic density in the brain.
Acta neuropathologica
Aoki N,Higashi S,Kawakami I,Kobayashi Z,Hosokawa M,Katsuse O,Togo T,Hirayasu Y,Akiyama H
Sat Sep 01 00:00:00 EDT 2012
Contrasting pathology of the stress granule proteins TIA-1 and G3BP in tauopathies.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Vanderweyde T,Yu H,Varnum M,Liu-Yesucevitz L,Citro A,Ikezu T,Duff K,Wolozin B
Wed Jun 13 00:00:00 EDT 2012
Contrasting pathology of the stress granule proteins TIA-1 and G3BP in tauopathies.
The Journal of neuroscience : the official journal of the Society for Neuroscience
Vanderweyde T,Yu H,Varnum M,Liu-Yesucevitz L,Citro A,Ikezu T,Duff K,Wolozin B
Wed Jun 13 00:00:00 EDT 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
Optineurin is colocalized with ubiquitin in Marinesco bodies.
Acta neuropathologica
Schwab C,Yu S,McGeer PL
Wed Feb 01 00:00:00 EST 2012
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
Motor neuron impairment mediated by a sumoylated fragment of the glial glutamate transporter EAAT2.
Glia
Foran E,Bogush A,Goffredo M,Roncaglia P,Gustincich S,Pasinelli P,Trotti D
Tue Nov 01 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
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
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
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
FUS pathology in basophilic inclusion body disease.
Acta neuropathologica
Munoz DG,Neumann M,Kusaka H,Yokota O,Ishihara K,Terada S,Kuroda S,Mackenzie IR
Sun Nov 01 00:00:00 EDT 2009