产品详情 |
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Antigenic Specificity | Ataxin-3 |
Clone | 1H9-2 |
Host Species | Mouse |
Reactive Species | human |
Isotype | IgG2a, κ |
Format | purified |
Size | 25 µg |
Concentration | n/a |
Applications | Western Blot (WB) |
Reviews / Ratings | If you have used this antibody, please help fellow researchers by submitting reviews to pAbmAbs and antYbuddY. |
Description | Purified anti-Ataxin-3. 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. |
Immunogen | Full length Ataxin-3 recombinant protein |
Other Names | ATX3, MJD, Machado-Joseph disease protein 1, SCA3, Spinocerebellar ataxia type 3 protein, JOS, josephin |
Gene, Accession # | Gene ID: 110616 |
Catalog # | 650401 |
Price | |
Order / More Info | Ataxin-3 Antibody from BIOLEGEND |
Product Specific References | PubMed: 23562578 |
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