21776-1-AP

antibody from Proteintech Group

Targeting: ATXN2 ATX2, SCA2, TNRC13

Western blot (Supportive data in Antibodypedia)

ELISA (Data presented on provider website)

Immunocytochemistry (Supportive data in Antibodypedia)

Immunoprecipitation (Data presented on provider website)

Immunohistochemistry (Supportive data in Antibodypedia)

Antibody Data

Product number

21776-1-AP

Provider

Proteintech Group

Proper citation

Proteintech Cat#21776-1-AP, RRID:AB_10858483

Product name

Ataxin 2 antibody

Antibody type

Polyclonal

Description

KD/KO validated Ataxin 2 antibody (Cat. #21776-1-AP) is a rabbit polyclonal antibody that shows reactivity with human, mouse, rat and has been validated for the following applications: IF, IHC, IP, WB, ELISA.

Reactivity

Human, Mouse, Rat

Host

Rabbit

Conjugate

Unconjugated

Isotype

IgG

Vial size

20ul, 150ul

References

Submitted references

A role for the cholinergic neuron circadian clock in RNA metabolism and mediating neurodegeneration.

Tam SB, Waldeck NJ, Wright M, Mojsilovic-Petrovic J, Baker EM, Kiskinis E, Bass J, Kalb RG
Life science alliance 2026 Mar;9(3)

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Therapeutic targeting of blood-derived protein infiltration to modulate neuroinflammation in cerebellar ataxia.

Park SM, Kim SS, Cho E, Lee GB, Lee JM, Kim DW, Kim JH, Kim JE, Choi GE, Lee HW, Suk K, Yoon JH, Kim S, Kim SR
Journal of neuroinflammation 2026 Apr 16;

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Spinal Cord Phosphoproteome of SCA2 Mouse Model Reveals Alteration of ATXN2-N-Term PRM-SH3-Actin Interactome and of Autophagy.

Almaguer-Mederos LE, Kandi AR, Sen NE, Canet-Pons J, Berger LM, Stokes MP, Abell K, Key J, Gispert S, Auburger G
Molecular & cellular proteomics : MCP 2025 Nov;24(11):101072

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AAV-based delivery of RNAi targeting ataxin-2 improves survival and pathology in TDP-43 mice.

Amado DA, Robbins AB, Whiteman KR, Smith AR, Chillon G, Chen Y, Fuller JA, Patty NA, Izda A, Cheng C, Nelson S, Dichter AI, Mazzoni EO, Monteys AM, Davidson BL
Nature communications 2025 Jun 25;16(1):5334

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ATXN2L primarily interacts with NUFIP2, the absence of ATXN2L results in NUFIP2 depletion, and the ATXN2-polyQ expansion triggers NUFIP2 accumulation.

Key J, Almaguer-Mederos LE, Kandi AR, Sen NE, Gispert S, Köpf G, Meierhofer D, Auburger G
Neurobiology of disease 2025 Jun 1;209:106903

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Intracerebellar upregulation of Rheb(S16H) ameliorates motor dysfunction in mice with SCA2.

Kim S, Park J, Eo H, Lee GB, Park SM, Shin M, Lee SE, Nam Y, Kim SR
Acta pharmacologica Sinica 2025 Jul;46(7):1852-1863

---

Intra-condensate demixing of TDP-43 inside stress granules generates pathological aggregates.

Yan X, Kuster D, Mohanty P, Nijssen J, Pombo-García K, Garcia Morato J, Rizuan A, Franzmann TM, Sergeeva A, Ly AM, Liu F, Passos PM, George L, Wang SH, Shenoy J, Danielson HL, Ozguney B, Honigmann A, Ayala YM, Fawzi NL, Dickson DW, Rossoll W, Mittal J, Alberti S, Hyman AA
Cell 2025 Jul 24;188(15):4123-4140.e18

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Influence of ATXN2 intermediate CAG repeats, 9bp duplication and alternative splicing on SCA3 pathogenesis.

Lauerer M, Faber J, Casadei N, Santana MM, Auburger G, Pogoda M, Admard J, Kaupp L, Kos PL, Raposo M, Lima M, de Almeida LP, Garcia-Moreno H, Giunti P, de Vries J, van de Warrenburg BP, van Gaalen J, Grobe-Einsler M, Koyak B, Reetz K, Erdlenbruch F, Jacobi H, Infante J, Hengel H, Schöls L, Klockgether T, Rieß O, Hübener-Schmid J, ESMI study group
Acta neuropathologica communications 2025 Jul 19;13(1):157

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Endogenous retrovirus-like proteins recruit UBQLN2 to stress granules and shape their functional biology.

Mohan HM, Fernandez MG, Huang C, Lin R, Ryou JH, Seyfried D, Grotewold N, Barget AJ, Whiteley AM, Basrur V, Mosalaganti S, Paulson HL, Sharkey LM
Science advances 2025 Jul 18;11(29):eadu6354

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Engineered microRNA scaffolds for potent gene silencing in vivo.

Militello G, Greig A, Bi C, Vasileva A, Zavodszky MI, Lo SC, Guilmette E, Clarner P, Liu B, Bhat G, Suh J, Dow L, Zuber J, Fellmann C, Premsrirut PK
Scientific reports 2025 Jul 1;15(1):21419

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TDP-43 Aggregate Seeding Impairs Autoregulation and Causes TDP-43 Dysfunction.

Mamede LD, Hu M, Titus AR, Vaquer-Alicea J, French RL, Diamond MI, Miller TM, Ayala YM
bioRxiv : the preprint server for biology 2025 Feb 12;

---

Liquid-liquid phase separation of ATXN2L enhances mRNA translation in hepatocellular carcinoma.

Wang S, Guo P, Ma W, Huang H, Zhang Q, Wolczynski S, Rahman N, Liu J, Tsang SY, Du X, Wang F, Gao J, Li X
Cell reports 2025 Dec 23;44(12):116588

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Fbxo42 promotes the degradation of Ataxin-2 granules to trigger terminal Xbp1 signaling.

Santos CC, Schweizer N, Cairrão F, Ramirez J, Osinalde N, Yang M, Gaspar CJ, Rasheva VI, Trigo ML, Hensel Z, Adrain C, Cordeiro TN, Voigt F, Gameiro PA, Mayor U, Domingos PM
Nature communications 2025 Aug 13;16(1):7523

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Context-dependent Interactors Regulate TDP-43 Dysfunction in ALS/FTLD.

Xie L, Zhu Y, Hurtle BT, Wright M, Robinson JL, Mauna JC, Brown EE, Ngo M, Bergmann CA, Xu J, Merjane J, Gleixner AM, Grigorean G, Liu F, Rossoll W, Lee EB, Kiskinis E, Chikina M, Donnelly CJ
bioRxiv : the preprint server for biology 2025 Apr 9;

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Deciphering the interactome of Ataxin-2 and TDP-43 in iPSC-derived neurons for potential ALS targets.

Tian Y, Heinsinger N, Hu Y, Lim UM, Wang Y, Fernandis AZ, Parmentier-Batteur S, Klein B, Uslaner JM, Smith SM
PloS one 2024;19(12):e0308428

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Endogenous retrovirus-like proteins recruit UBQLN2 to stress granules and alter their functional properties.

Mohan HM, Fernandez MG, Huang C, Lin R, Ryou JH, Seyfried D, Grotewold N, Whiteley AM, Barmada SJ, Basrur V, Mosalaganti S, Paulson HL, Sharkey LM
bioRxiv : the preprint server for biology 2024 Oct 25;

---

OptoProfilin: A Single Component Biosensor of Applied Cellular Stress.

Mann N, Hill J, Wang K, Hughes RM
Chembiochem : a European journal of chemical biology 2024 May 2;25(9):e202400007

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NS1 binding protein regulates stress granule dynamics and clearance by inhibiting p62 ubiquitination.

Jeon P, Ham HJ, Choi H, Park S, Jang JW, Park SW, Cho DH, Lee HJ, Song HK, Komatsu M, Han D, Jang DJ, Lee JA
Nature communications 2024 Dec 30;15(1):10925

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The effect of hormones insulin and glucagon on ubiquitin modifications elucidated by proteomics in liver cells.

Vemana HP, Dukhande VV
Life sciences 2023 Sep 15;329:121935

---

Mitigating a TDP-43 proteinopathy by targeting ataxin-2 using RNA-targeting CRISPR effector proteins.

Zeballos C MA, Moore HJ, Smith TJ, Powell JE, Ahsan NS, Zhang S, Gaj T
Nature communications 2023 Oct 14;14(1):6492

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TR-FRET-Based Immunoassay to Measure Ataxin-2 as a Target Engagement Marker in Spinocerebellar Ataxia Type 2.

Bux J, Sen NE, Klink IM, Hauser S, Synofzik M, Schöls L, Auburger G, Riess O, Hübener-Schmid J
Molecular neurobiology 2023 Jun;60(6):3553-3567

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Suboptimal Mitochondrial Activity Facilitates Nuclear Heat Shock Responses for Proteostasis and Genome Stability.

Park D, Yu Y, Kim JH, Lee J, Park J, Hong K, Seo JK, Lim C, Min KT
Molecules and cells 2023 Jun 30;46(6):374-386

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Poly(A)-binding protein is an ataxin-2 chaperone that regulates biomolecular condensates.

Boeynaems S, Dorone Y, Zhuang Y, Shabardina V, Huang G, Marian A, Kim G, Sanyal A, Şen NE, Griffith D, Docampo R, Lasker K, Ruiz-Trillo I, Auburger G, Holehouse AS, Kabashi E, Lin Y, Gitler AD
Molecular cell 2023 Jun 15;83(12):2020-2034.e6

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A spatially defined human Notch receptor interaction network reveals Notch intracellular storage and Ataxin-2-mediated fast recycling.

Bian W, Jiang H, Yao L, Hao W, Wu L, Li X
Cell reports 2023 Jul 25;42(7):112819

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Circadian clocks are modulated by compartmentalized oscillating translation.

Zhuang Y, Li Z, Xiong S, Sun C, Li B, Wu SA, Lyu J, Shi X, Yang L, Chen Y, Bao Z, Li X, Sun C, Chen Y, Deng H, Li T, Wu Q, Qi L, Huang Y, Yang X, Lin Y
Cell 2023 Jul 20;186(15):3245-3260.e23

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Diverse CMT2 neuropathies are linked to aberrant G3BP interactions in stress granules.

Cui Q, Bi H, Lv Z, Wu Q, Hua J, Gu B, Huo C, Tang M, Chen Y, Chen C, Chen S, Zhang X, Wu Z, Lao Z, Sheng N, Shen C, Zhang Y, Wu ZY, Jin Z, Yang P, Liu H, Li J, Bai G
Cell 2023 Feb 16;186(4):803-820.e25

---

OptoProfilin: A Single Component Biosensor of Applied Cellular Stress.

Mann N, Hill J, Wang K, Hughes RM
bioRxiv : the preprint server for biology 2023 Dec 19;

---

LONRF2 is a protein quality control ubiquitin ligase whose deficiency causes late-onset neurological deficits.

Li D, Johmura Y, Morimoto S, Doi M, Nakanishi K, Ozawa M, Tsunekawa Y, Inoue-Yamauchi A, Naruse H, Matsukawa T, Takeshita Y, Suzuki N, Aoki M, Nishiyama A, Zeng X, Konishi C, Suzuki N, Nishiyama A, Harris AS, Morita M, Yamaguchi K, Furukawa Y, Nakai K, Tsuji S, Yamazaki S, Yamanashi Y, Shimada S, Okada T, Okano H, Toda T, Nakanishi M
Nature aging 2023 Aug;3(8):1001-1019

---

Genome-wide CRISPR screen reveals v-ATPase as a drug target to lower levels of ALS protein ataxin-2.

Kim G, Nakayama L, Blum JA, Akiyama T, Boeynaems S, Chakraborty M, Couthouis J, Tassoni-Tsuchida E, Rodriguez CM, Bassik MC, Gitler AD
Cell reports 2022 Oct 25;41(4):111508

---

NUP62 localizes to ALS/FTLD pathological assemblies and contributes to TDP-43 insolubility.

Gleixner AM, Verdone BM, Otte CG, Anderson EN, Ramesh N, Shapiro OR, Gale JR, Mauna JC, Mann JR, Copley KE, Daley EL, Ortega JA, Cicardi ME, Kiskinis E, Kofler J, Pandey UB, Trotti D, Donnelly CJ
Nature communications 2022 Jun 13;13(1):3380

---

Saturated fatty acids entrap PDX1 in stress granules and impede islet beta cell function.

Zhang M, Yang C, Zhu M, Qian L, Luo Y, Cheng H, Geng R, Xu X, Qian C, Liu Y
Diabetologia 2021 May;64(5):1144-1157

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Atxn2-CAG100-KnockIn mouse spinal cord shows progressive TDP43 pathology associated with cholesterol biosynthesis suppression.

Canet-Pons J, Sen NE, Arsović A, Almaguer-Mederos LE, Halbach MV, Key J, Döring C, Kerksiek A, Picchiarelli G, Cassel R, René F, Dieterlé S, Fuchs NV, König R, Dupuis L, Lütjohann D, Gispert S, Auburger G
Neurobiology of disease 2021 May;152:105289

---

Hexanucleotide Repeat Expansions in c9FTD/ALS and SCA36 Confer Selective Patterns of Neurodegeneration In Vivo.

Todd TW, McEachin ZT, Chew J, Burch AR, Jansen-West K, Tong J, Yue M, Song Y, Castanedes-Casey M, Kurti A, Dunmore JH, Fryer JD, Zhang YJ, San Millan B, Teijeira Bautista S, Arias M, Dickson D, Gendron TF, Sobrido MJ, Disney MD, Bassell GJ, Rossoll W, Petrucelli L
Cell reports 2020 May 5;31(5):107616

---

Autism-Misregulated eIF4G Microexons Control Synaptic Translation and Higher Order Cognitive Functions.

Gonatopoulos-Pournatzis T, Niibori R, Salter EW, Weatheritt RJ, Tsang B, Farhangmehr S, Liang X, Braunschweig U, Roth J, Zhang S, Henderson T, Sharma E, Quesnel-Vallières M, Permanyer J, Maier S, Georgiou J, Irimia M, Sonenberg N, Forman-Kay JD, Gingras AC, Collingridge GL, Woodin MA, Cordes SP, Blencowe BJ
Molecular cell 2020 Mar 19;77(6):1176-1192.e16

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Mid-Gestation lethality of Atxn2l-Ablated Mice.

Key J, Harter PN, Sen NE, Gradhand E, Auburger G, Gispert S
International journal of molecular sciences 2020 Jul 20;21(14)

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LSM12-EPAC1 defines a neuroprotective pathway that sustains the nucleocytoplasmic RAN gradient.

Lee J, Park J, Kim JH, Lee G, Park TE, Yoon KJ, Kim YK, Lim C
PLoS biology 2020 Dec;18(12):e3001002

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Small-Molecule Modulation of TDP-43 Recruitment to Stress Granules Prevents Persistent TDP-43 Accumulation in ALS/FTD.

Fang MY, Markmiller S, Vu AQ, Javaherian A, Dowdle WE, Jolivet P, Bushway PJ, Castello NA, Baral A, Chan MY, Linsley JW, Linsley D, Mercola M, Finkbeiner S, Lecuyer E, Lewcock JW, Yeo GW
Neuron 2019 Sep 4;103(5):802-819.e11

---

Chronic optogenetic induction of stress granules is cytotoxic and reveals the evolution of ALS-FTD pathology.

Zhang P, Fan B, Yang P, Temirov J, Messing J, Kim HJ, Taylor JP
eLife 2019 Mar 20;8

---

Zika Virus Subverts Stress Granules To Promote and Restrict Viral Gene Expression.

Bonenfant G, Williams N, Netzband R, Schwarz MC, Evans MJ, Pager CT
Journal of virology 2019 Jun 15;93(12)

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Heterochromatin anomalies and double-stranded RNA accumulation underlie C9orf72 poly(PR) toxicity.

Zhang YJ, Guo L, Gonzales PK, Gendron TF, Wu Y, Jansen-West K, O'Raw AD, Pickles SR, Prudencio M, Carlomagno Y, Gachechiladze MA, Ludwig C, Tian R, Chew J, DeTure M, Lin WL, Tong J, Daughrity LM, Yue M, Song Y, Andersen JW, Castanedes-Casey M, Kurti A, Datta A, Antognetti G, McCampbell A, Rademakers R, Oskarsson B, Dickson DW, Kampmann M, Ward ME, Fryer JD, Link CD, Shorter J, Petrucelli L
Science (New York, N.Y.) 2019 Feb 15;363(6428)

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Aberrant deposition of stress granule-resident proteins linked to C9orf72-associated TDP-43 proteinopathy.

Chew J, Cook C, Gendron TF, Jansen-West K, Del Rosso G, Daughrity LM, Castanedes-Casey M, Kurti A, Stankowski JN, Disney MD, Rothstein JD, Dickson DW, Fryer JD, Zhang YJ, Petrucelli L
Molecular neurodegeneration 2019 Feb 15;14(1):9

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Generation of an Atxn2-CAG100 knock-in mouse reveals N-acetylaspartate production deficit due to early Nat8l dysregulation.

Sen NE, Canet-Pons J, Halbach MV, Arsovic A, Pilatus U, Chae WH, Kaya ZE, Seidel K, Rollmann E, Mittelbronn M, Meierhofer D, De Zeeuw CI, Bosman LWJ, Gispert S, Auburger G
Neurobiology of disease 2019 Dec;132:104559

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Antiviral Immune Response as a Trigger of FUS Proteinopathy in Amyotrophic Lateral Sclerosis.

Shelkovnikova TA, An H, Skelt L, Tregoning JS, Humphreys IR, Buchman VL
Cell reports 2019 Dec 24;29(13):4496-4508.e4

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Calcium-responsive transactivator (CREST) toxicity is rescued by loss of PBP1/ATXN2 function in a novel yeast proteinopathy model and in transgenic flies.

Park S, Park SK, Watanabe N, Hashimoto T, Iwatsubo T, Shelkovnikova TA, Liebman SW
PLoS genetics 2019 Aug;15(8):e1008308

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Internalization Characterization of Si Nanorod with Camouflaged Cell Membrane Proteins Reveals ATXN2 as a Negative Regulator.

Lu Y, Dai J, Kong N, Liu J, Gong J, Yao Y
Cells 2019 Aug 19;8(8)

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RNA Binding Antagonizes Neurotoxic Phase Transitions of TDP-43.

Mann JR, Gleixner AM, Mauna JC, Gomes E, DeChellis-Marks MR, Needham PG, Copley KE, Hurtle B, Portz B, Pyles NJ, Guo L, Calder CB, Wills ZP, Pandey UB, Kofler JK, Brodsky JL, Thathiah A, Shorter J, Donnelly CJ
Neuron 2019 Apr 17;102(2):321-338.e8

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Conserved Pbp1/Ataxin-2 regulates retrotransposon activity and connects polyglutamine expansion-driven protein aggregation to lifespan-controlling rDNA repeats.

Ostrowski LA, Hall AC, Szafranski KJ, Oshidari R, Abraham KJ, Chan JNY, Krustev C, Zhang K, Wang A, Liu Y, Guo R, Mekhail K
Communications biology 2018;1:187

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Atxn2 Knockout and CAG42-Knock-in Cerebellum Shows Similarly Dysregulated Expression in Calcium Homeostasis Pathway.

Halbach MV, Gispert S, Stehning T, Damrath E, Walter M, Auburger G
Cerebellum (London, England) 2017 Feb;16(1):68-81

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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.

Colombrita C, Onesto E, Megiorni F, Pizzuti A, Baralle FE, Buratti E, Silani V, Ratti A
The Journal of biological chemistry 2012 May 4;287(19):15635-47

Western blot

Supportive validation

Submitted by

Proteintech Group (provider)

Main image

Experimental details

HEK-293 cells were subjected to SDS PAGE followed by western blot with 21776-1-AP(ATXN2 antibody) at dilution of 1:500

Sample type

cell line

Immunocytochemistry

Supportive validation

Submitted by

Proteintech Group (provider)

Main image

Experimental details

Immunofluorescent analysis of 293 cells, using ATXN2 antibody 21776-1-AP at 1:25 dilution and Rhodamine-labeled goat anti-rabbit IgG (red).

Sample type

cell line

Immunohistochemistry

Supportive validation

Submitted by

Proteintech Group (provider)

Main image

Experimental details

Immunohistochemical of paraffin-embedded human gliomas using 21776-1-AP(ATXN2 antibody) at dilution of 1:50 (under 10x lens)

Sample type

tissue

Supportive validation

Submitted by

Proteintech Group (provider)

Main image

Experimental details

Immunohistochemical of paraffin-embedded human gliomas using 21776-1-AP(ATXN2 antibody) at dilution of 1:50 (under 40x lens)

Sample type

tissue