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You can also order by e-mail: info@biomol.com
Larger quantity required? Request bulk
You can also order by e-mail: info@biomol.com
Larger quantity required? Request bulk
Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant... more
Product information "Anti-ATXN3"
Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein encoded by this gene contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 12-44 to 52-86 is one cause of Machado-Joseph disease. There is a negative correlation between the age of onset and CAG repeat numbers. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. Protein function: Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates (PubMed:12297501, PubMed:17696782, PubMed:23625928, PubMed:28445460, PubMed:16118278). 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. Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription (PubMed:12297501). Regulates autophagy via the deubiquitination of 'Lys-402' of BECN1 leading to the stabilization of BECN1 (PubMed:28445460). [The UniProt Consortium]
Keywords: | Anti-ATX3, Anti-ATXN3, Anti-Ataxin-3, EC=3.4.19.12, Anti-Machado-Joseph disease protein 1, Anti-Spinocerebellar ataxia type 3 protein, ATXN3 Polyclonal Antibody |
Supplier: | Elabscience |
Supplier-Nr: | E-AB-60279 |
Properties
Application: | WB, IF |
Antibody Type: | Polyclonal |
Conjugate: | No |
Host: | Rabbit |
Species reactivity: | human |
Immunogen: | Recombinant protein of human ATXN3 |
Database Information
KEGG ID : | K11863 | Matching products |
UniProt ID : | P54252 | Matching products |
Gene ID | GeneID 4287 | Matching products |
Handling & Safety
Storage: | -20°C |
Shipping: | 4°C (International: -20°C) |
Caution
Our products are for laboratory research use only: Not for administration to humans!
Our products are for laboratory research use only: Not for administration to humans!
Information about the product reference will follow.
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