AB221143

Anti-Ataxin 3 antibody [EPR22418-147]

RabMAb
Recombinant
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(1 Publication)

Rabbit Recombinant Monoclonal Ataxin 3 antibody. Suitable for I-ELISA, WB and reacts with Recombinant fragment - Human, Mouse, Rat, Human samples. Cited in 1 publication.

View Alternative Names

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

3 Images

Indirect ELISA - Anti-Ataxin 3 antibody [EPR22418-147] (AB221143)

I-ELISA

Unknown

Indirect ELISA - Anti-Ataxin 3 antibody [EPR22418-147] (AB221143)

Indirect ELISA using ab221143 at varying antibody concentrations (1000-0 ng/ml) and Human ATXN3 antigen at 1000 ng/ml. Alkaline Phosphatase-conjugated AffiniPure Goat Anti-Rabbit IgG (H+L) at 1/2500 dilution was used as a secondary antibody.

Unknown

Western blot - Anti-Ataxin 3 antibody [EPR22418-147] (AB221143)

Blocking/diluting buffer and concentration : 5% NFDM/TBST

Exposure times :

Lane 1 : 10 seconds

Lanes 2-3 : 125 seconds

Lane 4 : 5 seconds

The full-length ataxin-3 (45kDa) and a 35-kDa band, likely to be an ataxin-3 isoform or fragment, are observed. The molecular weights are consistent with what have been described in the literature (PMID : 30455355 and UniProt annotation).

Lanes 1 - 3:

Western blot - Anti-Ataxin 3 antibody [EPR22418-147] (ab221143) at 1/1000 dilution

Lane 4:

Western blot - Anti-Ataxin 3 antibody [EPR22418-147] (ab221143) at 1/5000 dilution

Lane 1:

U-87 MG (human glioblastoma-astrocytoma epithelial cell line), whole cell lysate at 20 µg

Lane 2:

Human brain tissue lysate at 20 µg

Lane 3:

Human spleen tissue lysate at 20 µg

Lane 4:

Human cerebellum tissue lysate at 20 µg

Secondary

Lane 1:

Western blot - Goat Anti-Rabbit IgG H&L (HRP) (<a href='/en-us/products/secondary-antibodies/goat-rabbit-igg-h-l-hrp-ab97051'>ab97051</a>) at 1/100000 dilution

Lanes 2 - 4:

Western blot - VeriBlot for IP Detection Reagent (HRP) (<a href='/en-us/products/reagents/veriblot-for-ip-detection-reagent-hrp-ab131366'>ab131366</a>) at 1/1000 dilution

Predicted band size: 42 kDa

Observed band size: 35 kDa,45 kDa

false

Unknown

Western blot - Anti-Ataxin 3 antibody [EPR22418-147] (AB221143)

Blocking/diluting buffer and concentration : 5% NFDM/TBST

Exposure time :

Lanes 1-2 : 26 seconds

Lanes 3-4 : 3 minutes

Lane 5 : 15 seconds

Lane 6 : 3 minutes

Lane 7 : 59 seconds

The molecular weight is consistent with what has been described in the literature (PMID : 30455355).

All lanes:

Western blot - Anti-Ataxin 3 antibody [EPR22418-147] (ab221143) at 1/5000 dilution

Lane 1:

NIH/3T3 (mouse embryo fibroblast cell line), whole cell lysate at 20 µg

Lane 2:

Neuro-2a (mouse neuroblastoma cell line), whole cell lysate at 20 µg

Lane 3:

C6 (rat glial tumor glial cell), whole cell lysate at 20 µg

Lane 4:

Mouse skin tissue lysate at 20 µg

Lane 5:

Mouse spleen tissue lysate at 20 µg

Lane 6:

Rat brain tissue lysate at 20 µg

Lane 7:

Rat cerebellum tissue lysate at 20 µg

Secondary

All lanes:

Western blot - Goat Anti-Rabbit IgG H&L (HRP) (<a href='/en-us/products/secondary-antibodies/goat-rabbit-igg-h-l-hrp-ab97051'>ab97051</a>) at 1/100000 dilution

Predicted band size: 42 kDa

Observed band size: 45 kDa

false

Carrier free

Anti-Ataxin 3 antibody [EPR22418-147] - BSA and Azide free

Key facts

Host species

Rabbit

Clonality

Monoclonal

Clone number

EPR22418-147

Isotype

IgG

Carrier free

Reacts with

Mouse, Rat, Human

Applications

I-ELISA, WB

applications

Immunogen

The exact immunogen used to generate this antibody is proprietary information.

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Species", "Dilution Info", "Notes"], "tabs": { "all-applications": {"fullname" : "All Applications", "shortname": "All Applications"}, "IP" : {"fullname" : "Immunoprecipitation", "shortname":"IP"}, "FlowCyt" : {"fullname" : "Flow Cytometry", "shortname":"Flow Cyt"}, "IHCP" : {"fullname" : "Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)", "shortname":"IHC-P"}, "ICCIF" : {"fullname" : "Immunocytochemistry/ Immunofluorescence", "shortname":"ICC/IF"}, "IELISA" : {"fullname" : "Indirect ELISA", "shortname":"I-ELISA"}, "WB" : {"fullname" : "Western blot", "shortname":"WB"} }, "product-promise": { "all": "all", "testedAndGuaranteed": "tested", "guaranteed": "expected", "predicted": "predicted", "notRecommended": "not-recommended" } }, "values": { "Human": { "IP-species-checked": "notRecommended", "IP-species-dilution-info": "", "IP-species-notes": "", "FlowCyt-species-checked": "notRecommended", "FlowCyt-species-dilution-info": "", "FlowCyt-species-notes": "", "IHCP-species-checked": "notRecommended", "IHCP-species-dilution-info": "", "IHCP-species-notes": "", "ICCIF-species-checked": "notRecommended", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "", "IELISA-species-checked": "guaranteed", "IELISA-species-dilution-info": "", "IELISA-species-notes": "", "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "1/1000", "WB-species-notes": "" }, "Mouse": { "IP-species-checked": "notRecommended", "IP-species-dilution-info": "", "IP-species-notes": "", "FlowCyt-species-checked": "notRecommended", "FlowCyt-species-dilution-info": "", "FlowCyt-species-notes": "", "IHCP-species-checked": "notRecommended", "IHCP-species-dilution-info": "", "IHCP-species-notes": "", "ICCIF-species-checked": "notRecommended", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "", "IELISA-species-checked": "guaranteed", "IELISA-species-dilution-info": "", "IELISA-species-notes": "", "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "1/1000", "WB-species-notes": "" }, "Rat": { "IP-species-checked": "notRecommended", "IP-species-dilution-info": "", "IP-species-notes": "", "FlowCyt-species-checked": "notRecommended", "FlowCyt-species-dilution-info": "", "FlowCyt-species-notes": "", "IHCP-species-checked": "notRecommended", "IHCP-species-dilution-info": "", "IHCP-species-notes": "", "ICCIF-species-checked": "notRecommended", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "", "IELISA-species-checked": "guaranteed", "IELISA-species-dilution-info": "", "IELISA-species-notes": "", "WB-species-checked": "testedAndGuaranteed", "WB-species-dilution-info": "1/1000", "WB-species-notes": "" }, "Recombinant fragment - Human": { "IP-species-checked": "notRecommended", "IP-species-dilution-info": "", "IP-species-notes": "", "FlowCyt-species-checked": "notRecommended", "FlowCyt-species-dilution-info": "", "FlowCyt-species-notes": "", "IHCP-species-checked": "notRecommended", "IHCP-species-dilution-info": "", "IHCP-species-notes": "", "ICCIF-species-checked": "notRecommended", "ICCIF-species-dilution-info": "", "ICCIF-species-notes": "", "IELISA-species-checked": "testedAndGuaranteed", "IELISA-species-dilution-info": "1 µg/mL", "IELISA-species-notes": "", "WB-species-checked": "notRecommended", "WB-species-dilution-info": "", "WB-species-notes": "" } } }

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Product details

Patented technology
Our RabMAb^® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb^® patents.

What are the advantages of a recombinant monoclonal antibody?
This product is a recombinant monoclonal antibody, which offers several advantages including:

- High batch-to-batch consistency and reproducibility
- Improved sensitivity and specificity
- Long-term security of supply
- Animal-free batch production

For more information, read more on recombinant antibodies.

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Properties and storage information

Form

Liquid

Purification technique

Affinity purification Protein A

Storage buffer

pH: 7.2 - 7.4 Preservative: 0.01% Sodium azide Constituents: PBS, 40% Glycerol (glycerin, glycerine), 0.05% BSA

Shipped at conditions

Conditional Ambient

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 (1)

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

Molecular neurobiology 62:12768-12790 PubMed40450087

2025

Accurate Quantification of Mutant and Wild-Type polyQ Proteins Using Simple Western Capillary Immunoassays.

Applications

Unspecified application

Species

Unspecified reactive species

Bas Röttgering,Janwillem Testerink,Rudie Weij,Chantal Beekman,Nicole Datson

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'.

For licensing inquiries, please contact partnerships@abcam.com

Anti-Ataxin 3 antibody [EPR22418-147]

Indirect ELISA - Anti-Ataxin 3 antibody [EPR22418-147] (AB221143)

Western blot - Anti-Ataxin 3 antibody [EPR22418-147] (AB221143)

Western blot - Anti-Ataxin 3 antibody [EPR22418-147] (AB221143)

Related conjugates and formulations (1)

Key facts

Host species

Clonality

Clone number

Isotype

Carrier free

Reacts with

Applications

Immunogen

Alternative Products

Primary Antibodies

Complementary Products

Primary Antibodies

Proteins & Peptides

Primary Antibodies

Primary Antibodies

Reactivity data

Product details

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 (1)

Product promise