Recombinant Human Ataxin 3

• Cat.No.: ATXN3-478H
• Product Overview: Recombinant human Ataxin-3 was expressed inE.coliand purified by using conventional chromatography. MW =42.4 kDa (370aa).

Specification

• Cat. No.: ATXN3-478H
• Description: 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.
• Source: E.coli.
• Amino Acid Sequence: MESIFHEKQE GSLCAQHCLN NLLQGEYFSP VELSSIAHQL DEEERMRMAE GGVTSEDYRT FLQQPSGNMD DSGFFSIQVI SNALKVWGLE LILFNSPEYQ RLRIDPINER SFICNYKEHW FTVRKLGKQW FNLNSLLTGP ELISDTYLAL FLAQLQQEGY SIFVVKGDLP DCEADQLLQM IRVQQMHRPK LIGEELAQLK EQRVHKTDLE RVLEANDGSG MLDEDEEDLQ RALALSRQEI DMEDEEADLR RAIQLSMQGS SRNISQDMTQ TSGTNLTSEE LRKRREAYFE KQQQKQQQQQ QQQQQQQQQQ QQQQGDLSGQ SSHPCERPAT SSGALGSDLG DAMSEEDMLQ AAVTMSLETV RNDLKTEGKK
• Form: Liquid. In 20 mM Tris-HCl buffer (pH 7.5) containing 2mM DTT, 50mM NaCl, 10% glycerol.
• Purity: > 90% by SDS – PAGE.
• Concentration: 1 mg/ml (determined by Bradford assay).
• Endotoxin Level: < 1.0 EU per 1 μg of protein (determined by LAL method).
• Storage: Can be stored at +4°C short term (1-2 weeks). For long term storage, aliquot and store at -20°C or -70°C. Avoid repeated freezing and thawing cycles.

Gene Information

• Gene Name: ATXN3 ataxin 3 [ Homo sapiens ]
• Synonyms: ATXN3; ataxin 3; AT3; JOS; MJD; ATX3; MJD1; SCA3; josephin; olivopontocerebellar ataxia 3; Machado-Joseph disease protein 1; spinocerebellar ataxia type 3 protein; Machado-Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3); EC 3.1.2.15; Ataxin-3
• Gene ID: 4287
• mRNA Refseq: NM_001127696
• Protein Refseq: NP_001121168
• MIM: 607047
• UniProt ID: P54252
• Chromosome Location: 14q21
• Function: hydrolase activity; protein binding; ubiquitin thiolesterase activity