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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.
COA:
Hela cell nuclear extracts were resolved by electrophoresis, transferred to nitrocellulose, and probed with purified monoclonal anti-ataxin-3, clone 1H9-2, antibody. Proteins were visualized using an anti-mouse-IgG secondary conjugated to HRP and chemiluminescence detection.
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.
Other Names:
ATX3, MJD, Machado-Joseph disease protein 1, SCA3, Spinocerebellar ataxia type 3 protein, JOS, josephin
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.
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.
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This data display is provided for general comparisons between formats. Your actual data may vary due to variations in samples, target cells, instruments and their settings, staining conditions, and other factors. If you need assistance with selecting the best format contact our expert technical support team.
Purified anti-Ataxin-3
Hela cell nuclear extracts were resolved by electrophoresis, transferred to nitrocellulose, and probed with purified monoclonal anti-ataxin-3, clone 1H9-2, antibody. Proteins were visualized using an anti-mouse-IgG secondary conjugated to HRP and chemiluminescence detection.
