AB175265

Anti-Ataxin 3 antibody

Name: Anti-Ataxin 3 antibody (ab175265) | Abcam
Brand: Abcam Limited
SKU: AB175265
Price: 545 USD
Availability: InStock

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(7 Publications)

Rabbit Polyclonal Ataxin 3 antibody. Suitable for WB, IHC-P, ICC/IF and reacts with Human, Rat samples. Cited in 7 publications. Immunogen corresponding to Recombinant Full Length Protein corresponding to Human ATXN3.

View Alternative Names

ATX3, MJD, MJD1, SCA3, ATXN3, Ataxin-3, Machado-Joseph disease protein 1, Spinocerebellar ataxia type 3 protein

4 Images

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Ataxin 3 antibody (AB175265)

IHC-P

Unknown

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Ataxin 3 antibody (AB175265)

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of human lung cancer tissue labelling Ataxin 3 with ab175265 at 1/200. Magnification : 400x.

Immunocytochemistry/ Immunofluorescence - Anti-Ataxin 3 antibody (AB175265)

ICC/IF

Unknown

Immunocytochemistry/ Immunofluorescence - Anti-Ataxin 3 antibody (AB175265)

Immunocytochemistry/Immunofluorescence analysis of HeLa cells using ab175265. Blue DAPI for nuclear staining.

IHC-P

Unknown

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Ataxin 3 antibody (AB175265)

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) analysis of rat kidney tissue labelling Ataxin 3 with ab175265 at 1/200. Magnification : 400x.

Supplier Data

Western blot - Anti-Ataxin 3 antibody (AB175265)

All lanes:

Western blot - Anti-Ataxin 3 antibody (ab175265) at 1/500 dilution

All lanes:

MCF7 cell lysate

Predicted band size: 42 kDa

false

Key facts

Host species

Rabbit

Clonality

Polyclonal

Isotype

IgG

Carrier free

Reacts with

Rat, Human

Applications

IHC-P, ICC/IF, WB

applications

Immunogen

Recombinant Full Length Protein corresponding to Human ATXN3.

P54252

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "WB" : {"fullname" : "Western blot", "shortname":"WB"}, "IHCP" : {"fullname" : "Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)", "shortname":"IHC-P"}, "ICCIF" : {"fullname" : "Immunocytochemistry/ Immunofluorescence", "shortname":"ICC/IF"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "", "WB-species-notes": "", "IHCP-species-checked": "testedAndGuaranteed", "IHCP-species-dilution-info": "1/50 - 1/200", "IHCP-species-notes": "<a href='/en-us/products/primary-antibodies/rabbit-igg-polyclonal-isotype-control-chip-grade-ab171870'>ab171870</a> - Rabbit polyclonal IgG, is suitable for use as an isotype control with this antibody.", "ICCIF-species-checked": "testedAndGuaranteed", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "" }, "Rat": { "WB-species-checked": "guaranteed", "WB-species-dilution-info": "", "WB-species-notes": "", "IHCP-species-checked": "testedAndGuaranteed", "IHCP-species-dilution-info": "1/50 - 1/200", "IHCP-species-notes": "<a href='/en-us/products/primary-antibodies/rabbit-igg-polyclonal-isotype-control-chip-grade-ab171870'>ab171870</a> - Rabbit polyclonal IgG, is suitable for use as an isotype control with this antibody.", "ICCIF-species-checked": "guaranteed", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "" } } }

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Properties and storage information

Form

Liquid

Purification technique

Affinity purification Protein A

Storage buffer

pH: 7.3 Preservative: 0.02% Sodium azide Constituents: PBS, 50% Glycerol (glycerin, glycerine)

Shipped at conditions

Blue Ice

Appropriate short-term storage duration

1-2 weeks

Appropriate short-term storage conditions

+4°C

Appropriate long-term storage conditions

-20°C

Aliquoting information

Upon delivery aliquot

Storage information

Avoid freeze / thaw cycle

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Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

Ataxin 3 also known as ATXN3 is a protein that functions as a deubiquitinating enzyme. It plays an important role in protein homeostasis by editing ubiquitin chains on proteins marked for degradation. This protein is approximately 42 kDa in size and is expressed in various human tissues with higher expressions in the brain and testis. Ataxin 3 contains a polyglutamine (polyQ) tract which is responsible for its aggregation in certain pathologies.

Biological function summary

At the molecular level Ataxin 3 helps in regulating protein quality control by associating with the proteasome complex. It acts as a link between ubiquitination and protein degradation pathways. The involvement of Ataxin 3 in transcriptional regulation and cytoskeletal organization further highlights its importance in cellular health. Its deubiquitinating activity implies its participation in recycling ubiquitins which are important to maintaining ubiquitin pools within the cell.

Pathways

Ataxin 3 is embedded within the ubiquitin-proteasome system which is essential for protein degradation. It links with pathways such as the protein quality control and endocytosis pathways. Ataxin 3 interacts with proteins like parkin and Ubiquitin C-terminal hydrolase L1 (UCH-L1) both of which are important for removing damaged or misfolded proteins and in the prevention of protein accumulation. These interactions show its broad role in cell maintenance and preventing toxic accumulations.

The malfunction of Ataxin 3 directly relates to Spinocerebellar Ataxia Type 3 (SCA3) also known as Machado-Joseph Disease. The elongated polyQ tract within Ataxin 3 leads to its aggregation resulting in neuronal death. This aggregation process is shared with proteins such as Ataxin 2 which also forms aggregates in certain neurological conditions indicating a pathogenic overlap with related neurodegenerative diseases. Understanding Ataxin 3’s role offers insights into therapeutic targets for these conditions.

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Product protocols

For this product, it's our understanding that no specific protocols are required. You can visit:

Visit the General protocols
Visit the Troubleshooting

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Target data

Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates (PubMed : 12297501, PubMed : 16118278, PubMed : 17696782, PubMed : 23625928, PubMed : 28445460, PubMed : 33157014). Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins (PubMed : 17696782). Involved in degradation of misfolded chaperone substrates via its interaction with STUB1/CHIP : recruited to monoubiquitinated STUB1/CHIP, and restricts the length of ubiquitin chain attached to STUB1/CHIP substrates and preventing further chain extension (By similarity). Interacts with key regulators of transcription and represses transcription : acts as a histone-binding protein that regulates transcription (PubMed : 12297501). Acts as a negative regulator of mTORC1 signaling in response to amino acid deprivation by mediating deubiquitination of RHEB, thereby promoting RHEB inactivation by the TSC-TBC complex (PubMed : 33157014). Regulates autophagy via the deubiquitination of 'Lys-402' of BECN1 leading to the stabilization of BECN1 (PubMed : 28445460).

See full target information ATXN3

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Publications (7)

Recent publications for all applications. Explore the full list and refine your search

Gene therapy 32:462-474 PubMed40721863

2025

Gene editing for Spinocerebellar ataxia type 3 taking advantage of the human ATXN3L paralog as replacement gene.

Applications

Unspecified application

Species

Unspecified reactive species

Margareta Rybarikova,Maria Rey,Ed Hasanovic,Mélanie Sipion,Lukas Rambousek,Nicole Déglon

Antioxidants (Basel, Switzerland) 13: PubMed39765823

2025

Erinacine A-Enriched Mycelium Ethanol Extract Lessens Cellular Damage in Cell and Models of Spinocerebellar Ataxia Type 3 by Improvement of Nrf2 Activation.

Applications

Unspecified application

Species

Unspecified reactive species

Yu-Ling Wu,Hai-Lun Sun,Jui-Chih Chang,Wei-Yong Lin,Pei-Yin Chen,Chin-Chu Chen,Li-Ya Lee,Chien-Chun Li,Mingli Hsieh,Haw-Wen Chen,Ya-Chen Yang,Chin-San Liu,Kai-Li Liu

Scientific reports 14:25979 PubMed39472629

2024

Astragaloside IV reduces mutant Ataxin-3 levels and supports mitochondrial function in Spinocerebellar Ataxia Type 3.

Applications

Unspecified application

Species

Unspecified reactive species

Yongshiou Lin,Wenling Cheng,Juichih Chang,Yuling Wu,Mingli Hsieh,Chinsan Liu

Antioxidants (Basel, Switzerland) 11: PubMed35883884

2022

In Vitro Efficacy and Molecular Mechanism of Curcumin Analog in Pathological Regulation of Spinocerebellar Ataxia Type 3.

Applications

Unspecified application

Species

Unspecified reactive species

Yu-Ling Wu,Jui-Chih Chang,Yi-Chun Chao,Hardy Chan,Mingli Hsieh,Chin-San Liu

Biomedicines 10: PubMed35203722

2022

IGF-1 as a Potential Therapy for Spinocerebellar Ataxia Type 3.

Applications

Unspecified application

Species

Unspecified reactive species

Yong-Shiou Lin,Wen-Ling Cheng,Jui-Chih Chang,Ta-Tsung Lin,Yi-Chun Chao,Chin-San Liu

Stem cells and development 27:756-770 PubMed29661116

2018

CRISPR/Cas9-Targeted Deletion of Polyglutamine in Spinocerebellar Ataxia Type 3-Derived Induced Pluripotent Stem Cells.

Applications

Unspecified application

Species

Unspecified reactive species

Shuming Ouyang,Yingjun Xie,Zeyu Xiong,Yi Yang,Yexing Xian,Zhanhui Ou,Bing Song,Yuchang Chen,Yuhuan Xie,Haoxian Li,Xiaofang Sun

PloS one 12:e0171127 PubMed28182673

2017

The expanded CAG repeat in the huntingtin gene as target for therapeutic RNA modulation throughout the HD mouse brain.

Applications

Unspecified application

Species

Unspecified reactive species

Nicole A Datson,Anchel González-Barriga,Eleni Kourkouta,Rudie Weij,Jeroen van de Giessen,Susan Mulders,Outi Kontkanen,Taneli Heikkinen,Kimmo Lehtimäki,Judith C T van Deutekom

View all publications

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Product promise

We are committed to supporting your work with high-quality reagents, and we're here for you every step of the way. In the unlikely event that one of our products does not perform as expected, you're protected by our Product Promise.

For full details, please see our Terms & Conditions

Please note: All products are 'FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC OR THERAPEUTIC PROCEDURES'.

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Anti-Ataxin 3 antibody

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Ataxin 3 antibody (AB175265)

Immunocytochemistry/ Immunofluorescence - Anti-Ataxin 3 antibody (AB175265)

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Ataxin 3 antibody (AB175265)

Western blot - Anti-Ataxin 3 antibody (AB175265)

Key facts

Host species

Clonality

Isotype

Carrier free

Reacts with

Applications

Immunogen

Alternative Products

Primary Antibodies

Primary Antibodies

Complementary Products

Secondary Antibodies

Primary Antibodies

Primary Antibodies

Primary Antibodies

Reactivity data

Properties and storage information

Form

Purification technique

Storage buffer

Shipped at conditions

Appropriate short-term storage duration

Appropriate short-term storage conditions

Appropriate long-term storage conditions

Aliquoting information

Storage information

Supplementary information

Biological function summary

Pathways

Product protocols

Target data

Publications (7)

Product promise