Ataxin 3 Recombinant Protein | ATXN3 recombinant protein

Ataxin 3, 1-370aa, Human, E Coli

Cat. Num. AAA203345

• Gene Names: ATXN3; AT3; JOS; MJD; ATX3; MJD1; SCA3
• Applications: SDS-Page
• Purity: > 90% by SDS-PAGE
• Synonyms: Ataxin 3; 1-370aa; Human; E Coli; ATX3; MJD; MJD1; SCA3; ATXN3; JOS; AT3; EC 3.4.22.; Josephin; Machado Joseph disease; Machado Joseph disease protein 1; Machado-Joseph disease protein 1 homolog; MJD gene; Rsca3; SCA3 gene; Spinocerebellar ataxia type 3 protein; ; ATXN3 recombinant protein

• Host: E Coli
• Purity/Purification: > 90% by SDS-PAGE
• Form/Format: Liquid. In 20 mM Tris-HCl Buffer (pH 7.5) containing 2mM DTT, 50mM NaCl, 10% glycerol.
• Concentration: 1 mg/ml (determined by Bradford assay) (varies by lot)
• Sequence Length: 370

• Applicable Applications for ATXN3 recombinant protein: SDS-PAGE
• Antigen Species: Human
• Tag: Non-tagged
• Endotoxin Level: < 1.0 EU per 1 microgram of protein (determined by LAL method)
• Preparation and Storage: Can be stored 2-8°C for 1 week. For long term storage aliquot and store at -20°C to -80°C. Avoid freeze thaw cycles.

SDS-Page

(3ug by SDS-PAGE under reducing condtion and visualized by coomassie blue stain)

Related Product Information for ATXN3 recombinant protein

Ataxin 3 is also known as Machado-Joseph disease protein 1. Machado-Joseph disease is one of several hereditary autosomal dominant neurodegenerative disorders. This protein contains trinucleotide CAG repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is the cause of Machado-Joseph disease. Ataxin 3 interacts with the major histone acetyltransferases cAMP-response-element binding protein (CREB)-binding protein, p300, and p300/CREB-binding protein-associated factor and inhibits transcription by these coactivators. Also, ataxin-3 is a proteasome-associated factor that mediates the degradation of ubiquitinated proteins. Recombinant human Ataxin-3 was expressed in E Coli and purified by using conventional chromatography.

Product Categories/Family for ATXN3 recombinant protein

Transcription & Translation

References

Doss Pepe EW., et al. (2003). Mol Cell Biol. 23 (18):6469-83. ; Paulson., et al. (1997). Ann Neurol. 41 (4):453-62.;

NCBI and Uniprot Product Information

• NCBI GI #: 21708052
• NCBI GeneID: 4287
• Molecular Weight: 42.4 kDa (370aa), confirmed by MALDI-TOF.
• NCBI Official Full Name: ATXN3 protein
• NCBI Official Synonym Full Names: ataxin 3
• NCBI Official Symbol: ATXN3
• NCBI Official Synonym Symbols: AT3; JOS; MJD; ATX3; MJD1; SCA3
• NCBI Protein Information: ataxin-3
• UniProt Protein Name: Ataxin-3
• Protein Family: Ataxin
• UniProt Gene Name: ATXN3
• UniProt Synonym Gene Names: ATX3; MJD; MJD1; SCA3

NCBI Description

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. [provided by RefSeq, Jul 2016]

Uniprot Description

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).