- Clone
- 1H9-2 (See other available formats)
- Regulatory Status
- RUO
- Other Names
- ATX3, ATXN3, MJD, Machado-Joseph disease protein 1, SCA3, Spinocerebellar ataxia type 3 protein, JOS, josephin
- Isotype
- Mouse IgG2a, κ
- Ave. Rating
- Submit a Review
- Product Citations
- publications
Cat # | Size | Price | Quantity Check Availability | Save | ||
---|---|---|---|---|---|---|
650401 | 25 µg | 100€ | ||||
650402 | 100 µg | 212€ |
Ataxin-3 is an ubiquitously expressed protein which contains a poly-glutamine tract. Genetically abnormal expansion of the poly-glutamine repeats results in accumulation of ubiquitinated ataxin-3 protein aggregates and leads to a neurodegenerative disorder, Machado–Joseph disease (MJD). The protein has ubiquitin interacting motifs and a N-terminal Cysteine residue (C14) responsible for its deubiquitinating activity. Ataxin-3 binds and hydrolyzes polyubiquitinated proteins, and interacts with key proteasome components, such as p97/VCP and p45, indicating that Ataxin-3 may play functional roles in ubiquitin-proteasome system. The protein also act as a transcriptional repressor when interacting with transcriptional activators and coactivators.
Product DetailsProduct Details
- Verified Reactivity
- Human
- Antibody Type
- Monoclonal
- Host Species
- Mouse
- Immunogen
- Full length Ataxin-3 recombinant protein
- Formulation
-
This antibody is provided in phosphate-buffered solution, pH 7.2, containing 0.09% sodium azide.
Previous lots of this product may have been formulated with 0.1% or 0.05% NaN3 instead of 0.09% NaN3. For further information please contact BioLegend Technical Support or Customer Service. - Preparation
- The antibody was purified by affinity chromatography.
- Concentration
- 0.5 mg/ml
- Storage & Handling
- Upon receipt, store undiluted between 2°C and 8°C.
- Application
-
WB - Quality tested
- Recommended Usage
-
Each lot of this antibody is quality control tested by Western blotting. Western blotting, suggested working dilution(s): Use 0.5 µg per ml antibody dilution buffer for each mini-gel. It is recommended that the reagent be titrated for optimal performance for each application.
-
Application References
(PubMed link indicates BioLegend citation) -
- Zhou L, et al. 2013. Neuroscience. 23:243. PubMed
- Product Citations
-
- RRID
-
AB_10900236 (BioLegend Cat. No. 650401)
AB_10900236 (BioLegend Cat. No. 650402)
Antigen Details
- Structure
- 364 aa with predicted molecular weight of 42 kD. The protein contains highly polymorphic polyglutamine repeats and has two isoforms which contain 2 or 3 ubiquitin interacting motifs (UIMs).
- Distribution
-
Nuclear
- Function
- Has transcriptional repression activity through interaction with regulators of transcription and histones. It regulates ER-associated protein degradation and autophagy when interacts with p97/VCP. It also contains deubiquitination activity.
- Interaction
- Ataxin-3 interacts with ubiquitin, p97/VCP, histone acetyl-transferases (p300, CBP, and PCAF), and histone.
- Biology Area
- Cell Biology, Chromatin Remodeling/Epigenetics, Transcription Factors, Ubiquitin/Protein Degradation
- Antigen References
-
1. Kawaguchi Y, et al. 1994. Nat. Genet. 3:213.
2. Mao Y, et al. 2005. P. Natl. Acad. Sci. USA 102:12700.
3. Li F, et al. 2002. J. Biol. Chem. 277:45004.
4. Zhong X, et al. 2006. Hum. Mol. Genet. 15:2409.
5. Tait D, et al. 1998. Hum. Mol. Genet. 7:991. - Gene ID
- 110616 View all products for this Gene ID
- UniProt
- View information about Ataxin-3 on UniProt.org
Related Pages & Pathways
Pages
Related FAQs
Other Formats
View All Ataxin-3 Reagents Request Custom ConjugationDescription | Clone | Applications |
---|---|---|
Purified anti-Ataxin-3 | 1H9-2 | WB |
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Purified anti-Ataxin-3
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