protein

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

MBS203345

0.05 mg

310 USD

Recombinant Protein

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

Ataxin 3

Ataxin 3; ATX3; MJD; MJD1; SCA3; ATXN3; JOS; ATXN3; 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 protein; Ataxin-3; ataxin-3; Machado-Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3); Machado-Joseph disease protein 1; ataxin 3 variant h; ataxin 3 variant m; ataxin 3 variant ref; josephin; spinocerebellar ataxia type 3 protein; ataxin 3; Machado-Joseph disease protein 1; Spinocerebellar ataxia type 3 protein

ATXN3; ATXN3; AT3; JOS; MJD; ATX3; MJD1; SCA3; ATX3; MJD; MJD1; SCA3

ATX3_HUMAN

370

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

> 90% by SDS - PAGE

Liquid. In 20 mM Tris-HCl buffer (pH 7.5) containing 2mM DTT, 50mM NaCl, 10% glycerol.

1 mg/ml (determined by Bradford assay)

Can be stored at 4 degree C short term (1-2 weeks). For long term storage, aliquot and store at -20 degree C or -70 degree C. Avoid repeated freezing and thawing cycles.

SDS-PAGE

Antigen Species: Human

Expression System: E Coli. Endotoxin: < 1.0 Eu per 1 microgram of protein (determined by LAL method)

Transcription & Translation

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.

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

21708052

AAH33711

42.4 kDa (370aa), confirmed by MALDI-TOF.

Protein Processing In Endoplasmic Reticulum Pathway 167325!!Protein Processing In Endoplasmic Reticulum Pathway 167190

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 13-36 to 68-79 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, Sep 2009]

0.05 mg

310 USD