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| Product Name | Ataxin 3 (ATXN3) protein (His tag) |
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| Description | Ataxin-3, also known as Machado-Joseph Disease (MJD) Protein 1 and Spinocerebellar Ataxia Type 3, is a 364 amino acid (aa), ubiquitously expressed cytoplasmic and nuclear protein with a predicted molecular weight of 42 kDa. Ataxin-3 functions as a deubiquitinating enzyme. Human Ataxin-3 shares 87% and 86% aa sequence identity with mouse and rat Ataxin-3, respectively. Full-length Ataxin-3 contains a N-terminal josephin domain, two ubiquitin interacting motifs, and a variable C-terminus consisting of a polyglutamine stretch and tail. As a deubiquitinating enzyme, Ataxin-3 plays a critical role in affecting the ubiquitination status of proteins for quality control and other cellular pathways. In turn, the ubiquitination of Ataxin-3 was shown to enhance its capacity to cleave ubiquitin chains. By opposing the actions of the ubiquitin-conjugating (E2) enzyme UBE2W, Ataxin-3 is believed to control the activity of the ubiquitin ligase (E3) C-terminus of Hsp70 Interacting Protein (CHIP). CHIP binds to protein chaperones and represents an important molecular link between the chaperone and ubiquitin-proteasome system. Expression of murine Ataxin-3 is thought to be important for myogenesis, an effect that is dependent on the regulation of Integrin alpha 5 levels. Mutations in the ATXN3 gene are the cause of MJD, also known as spinocerebellar ataxia 3, an autosomal dominant neurodegenerative disorder characterized by nuclear aggregation of Ataxin-3Molecules featuring an expanded polyglutamine trac. |
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| Size | 50 µg |
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| Concentration | n/a |
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| Applications | SDS-PAGE (SDS) |
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| Other Names | Mjd|zgc:56323|ATXN3|at3|jos|mjd|atx3|mjd1|sca3|ataxin-3|MJD1|MGC83584|AT3|ATX3|JOS|MJD|SCA3|2210008M02Rik|AI463012|AI647473|Sca3|Rsca3 |
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| Gene, Accession, CAS # | Gene ID: 4287 |
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| Catalog # | ABIN2745607 |
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| Order / More Info | Ataxin 3 (ATXN3) protein (His tag) from ANTIBODIES-ONLINE GmbH |
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| Product Specific References | n/a |
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