Proteintech

TDP-43 Monoclonal Antibody (6H6E12)

Product Image Gallery

TDP-43 Antibody in Immunohistochemistry (Paraffin) (IHC (P)) — Immunohistochemistry of paraffin-embedded human pancreas cancer tissue slide using 60019-2-Ig (TDP-43 (human specific) antibody) at dilution of 1:10000 (under 40x lens) heat mediated antigen retrieved with Tris-EDTA buffer (pH 9).

TDP-43 Antibody in Immunohistochemistry (IHC) — 40X of FTLD-U case stained by 10782-2-AP and 60019-2-IG, showing dystrophic neurites. (Figs were provided by Linda K. Kwong).

TDP-43 Antibody in Western Blot (WB) — Various cells were subjected to SDS PAGE followed by western blot with 60019-2-IG (TDP-43 (human specific) antibody) at dilution of 1:50000 incubated at room temperature for 1.5 hours.

TDP-43 Antibody in Flow Cytometry (Flow) — 1X10^6 MCF-7 cells were stained with 0.2ug TDP-43 (human specific) antibody (60019-2-IG, red) and control antibody (blue). Fixed with 90% MeOH blocked with 3% BSA (30 min). Alexa Fluor 488-conjugated AffiniPure Goat Anti-Mouse IGG (H+L) with dilution 1:1500.

TDP-43 Antibody in Immunoprecipitation (IP) — IP result of anti-TDP-43 (human specific) (IP:60019-2-IG, 5ug; Detection:60019-2-IG 1:1000) with K-562 cells lysate 1720ug.

Product Details

60019-2-IG

Applications	Tested Dilution	Publications
Western Blot (WB)	1:5,000-1:100,000	View 22 publications 22 publications
Immunohistochemistry (IHC)	1:50-1:50,000	View 13 publications 13 publications
Immunocytochemistry (ICC/IF)	-	View 10 publications 10 publications
Flow Cytometry (Flow)	0.2 µg/1x10^6 cells	-
Immunoprecipitation (IP)	0.5-4.0 µg	View 3 publications 3 publications
Miscellaneous PubMed (Misc)	-	View 3 publications 3 publications

Product Specifications
Species Reactivity	Human
Published species	Human
Host/Isotype	Mouse / IgG1
Class	Monoclonal
Type	Antibody
Clone	6H6E12
Immunogen	TDP-43 (human specific) Fusion Protein Ag1231
Conjugate	Unconjugated
Form	Liquid
Concentration	1.07 mg/mL
Purification	Caprylic acid precipitation
Storage buffer	PBS, pH 7.3, with 50% glycerol
Contains	0.1% sodium azide
Storage conditions	-20°C

Product Specific Information

60019-2-Ig is a mouse monoclonal antibody recognizing the cleavage product of 20-30 kDa in addition to the native and phosphorylated forms of TDP-43. Immunohistochemical analyses of TDP-43 using this antibody detect both normal diffuse nuclear staining and insoluble inclusions in pathologic tissues. Notably this antibody only recognizes human TDP-43 but not reacts with mouse or rat TDP-43.

Target Information

TDP43 was first identified as a novel cellular protein that binds to HIV-1 virus TAR DNA sequence motifs and acts a transcriptional repressor to the HIV-1 LTR. Later experiments revealed that TDP43 also regulates the splicing of exon 9 of the cystic fibrosis transmembrane conductance regular (CFTR), most likely through the association with the UG repeats at the 3"e; splice site. A hyperphosphorylated, ubiquitinated, and cleaved form of TDP43 known as pathologic TDP43 is the major disease protein in ubiquitin-positive, tau-, and alpha-synuclein-negative frontotemporal dementia (FLTD-U). TDP43 is not related to TRBP1, and RNA binding protein that binds HIV-1 TAR RNA sequences.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.

Product References

Early Cognitive/Social Deficits and Late Motor Phenotype in Conditional Wild-Type TDP-43 Transgenic Mice.

Frontiers in aging neuroscience

Alfieri JA,Silva PR,Igaz LM

Thu Oct 01 00:00:00 EDT 2020

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

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

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

Split GFP technologies to structurally characterize and quantify functional biomolecular interactions of FTD-related proteins.

Scientific reports

Foglieni C,Papin S,Salvadè A,Afroz T,Pinton S,Pedrioli G,Ulrich G,Polymenidou M,Paganetti P

Wed Oct 25 00:00:00 EDT 2017

Synapse Dysfunction of Layer V Pyramidal Neurons Precedes Neurodegeneration in a Mouse Model of TDP-43 Proteinopathies.

Cerebral cortex (New York, N.Y. : 1991)

Handley EE,Pitman KA,Dawkins E,Young KM,Clark RM,Jiang TC,Turner BJ,Dickson TC,Blizzard CA

Sat Jul 01 00:00:00 EDT 2017

Progranulin regulates lysosomal function and biogenesis through acidification of lysosomes.

Human molecular genetics

Tanaka Y,Suzuki G,Matsuwaki T,Hosokawa M,Serrano G,Beach TG,Yamanouchi K,Hasegawa M,Nishihara M

Wed Mar 01 00:00:00 EST 2017

Progranulin regulates lysosomal function and biogenesis through acidification of lysosomes.

Human molecular genetics

Tanaka Y,Suzuki G,Matsuwaki T,Hosokawa M,Serrano G,Beach TG,Yamanouchi K,Hasegawa M,Nishihara M

Wed Mar 01 00:00:00 EST 2017

ALS/FTLD: experimental models and reality.

Acta neuropathologica

Tan RH,Ke YD,Ittner LM,Halliday GM

Wed Feb 01 00:00:00 EST 2017

Gain-of-function profilin 1 mutations linked to familial amyotrophic lateral sclerosis cause seed-dependent intracellular TDP-43 aggregation.

Human molecular genetics

Tanaka Y,Nonaka T,Suzuki G,Kametani F,Hasegawa M

Fri Apr 01 00:00:00 EDT 2016

Gain-of-function profilin 1 mutations linked to familial amyotrophic lateral sclerosis cause seed-dependent intracellular TDP-43 aggregation.

Human molecular genetics

Tanaka Y,Nonaka T,Suzuki G,Kametani F,Hasegawa M

Fri Apr 01 00:00:00 EDT 2016

Amyotrophic lateral sclerosis and denervation alter sphingolipids and up-regulate glucosylceramide synthase.

Human molecular genetics

Henriques A,Croixmarie V,Priestman DA,Rosenbohm A,Dirrig-Grosch S,D'Ambra E,Huebecker M,Hussain G,Boursier-Neyret C,Echaniz-Laguna A,Ludolph AC,Platt FM,Walther B,Spedding M,Loeffler JP,Gonzalez De Aguilar JL

Sun Dec 20 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

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

Evidence of a link between ubiquilin 2 and optineurin in amyotrophic lateral sclerosis.

Human molecular genetics

Osaka M,Ito D,Yagi T,Nihei Y,Suzuki N

Sun Mar 15 00:00:00 EDT 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

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

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

Reversible behavioral phenotypes in a conditional mouse model of TDP-43 proteinopathies.

The Journal of neuroscience : the official journal of the Society for Neuroscience

Alfieri JA,Pino NS,Igaz LM

Wed Nov 12 00:00:00 EST 2014

Distinct pathways leading to TDP-43-induced cellular dysfunctions.

Human molecular genetics

Yamashita M,Nonaka T,Hirai S,Miwa A,Okado H,Arai T,Hosokawa M,Akiyama H,Hasegawa M

Fri Aug 15 00:00:00 EDT 2014

Prevention of intestinal obstruction reveals progressive neurodegeneration in mutant TDP-43 (A315T) mice.

Molecular neurodegeneration

Herdewyn S,Cirillo C,Van Den Bosch L,Robberecht W,Vanden Berghe P,Van Damme P

Tue Jun 17 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

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

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

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

An autopsy case of sporadic amyotrophic lateral sclerosis associated with the I113T SOD1 mutation.

Neuropathology : official journal of the Japanese Society of Neuropathology

Nakamura S,Wate R,Kaneko S,Ito H,Oki M,Tsuge A,Nagashima M,Asayama S,Fujita K,Nakamura M,Maruyama H,Kawakami H,Kusaka H

Sat Feb 01 00:00:00 EST 2014

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

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

Prion-like properties of pathological TDP-43 aggregates from diseased brains.

Cell reports

Nonaka T,Masuda-Suzukake M,Arai T,Hasegawa Y,Akatsu H,Obi T,Yoshida M,Murayama S,Mann DM,Akiyama H,Hasegawa M

Thu Jul 11 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

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

Cytosolic 5'-nucleotidase 1A autoimmunity in sporadic inclusion body myositis.

Annals of neurology

Larman HB,Salajegheh M,Nazareno R,Lam T,Sauld J,Steen H,Kong SW,Pinkus JL,Amato AA,Elledge SJ,Greenberg SA

Fri Mar 01 00:00:00 EST 2013

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

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

Inclusion body myositis coexisting with hypertrophic cardiomyopathy: an autopsy study.

Neuromuscular disorders : NMD

Inamori Y,Higuchi I,Inoue T,Sakiyama Y,Hashiguchi A,Higashi K,Shiraishi T,Okubo R,Arimura K,Mitsuyama Y,Takashima H

Wed Aug 01 00:00:00 EDT 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

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

Dysfunction of the ubiquitin-proteasome system in the cerebellum of aging Ts65Dn mice.

Experimental neurology

Necchi D,Lomoio S,Scherini E

Thu Dec 01 00:00:00 EST 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

AMSH is required to degrade ubiquitinated proteins in the central nervous system.

Biochemical and biophysical research communications

Suzuki S,Tamai K,Watanabe M,Kyuuma M,Ono M,Sugamura K,Tanaka N

Fri May 20 00:00:00 EDT 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

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

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

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