Recombinant Human Ataxin 3 Protein

重组人His6-Ataxin-3蛋白 | 重组人共济失调蛋白3

Ataxin-3 | Machado-Joseph disease protein 1 | Spinocerebellar ataxia type 3 protein | ATXN3 | ATX3 | MJD | MJD1 | SCA3 | AT3 | JOS

rp184114-10μg

10μg

无载体

≥95%(SDS-PAGE)

Ataxin 3 is otherwise known as Machado-Joseph disease protein 1. Machado–Joseph disease is a hereditary autosomal dominant neurodegenerative disorder. ATXN3 contains trinucleotide CAG repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 causes the Machado-Joseph disease. ATXN3 is a poly-ubiquitin-binding protein whose cellular turnover is regulated by its catalytic activity. In addition, ATXN3 is a proteasome-associated factor which mediates the degradation of ubiquitinated proteins. ATXN3 folds reversibly using a single intermediate; partial destabilization of ATXN3 by chemical denaturation causes the formation of fibrillar aggregates by the non-pathological variant. 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 hinders transcription by these coactivators. Post-translational: Monoubiquitinated N-terminally by UBE2W, possibly leading to activate the deubiquitinating enzyme activity (PubMed:23696636).

Immobilized Recombinant Human Ataxin 3 Protein (rp184114) at 1.0 μg/mL can bind Recombinant Ataxin 3 Antibody (Ab089900) with the EC50 of 2.1 ng/mL.

重组表达

42.1 kDa

C-His

E. coli Accession #: P54252 | E. coli

<1.0 EU/μg

人(Human)

1-361 aa

MESIFHEKQEGSLCAQHCLNNLLQGEYFSPVELSSIAHQLDEEERMRMAEGGVTSEDYRTFLQQPSGNMDDSGFFSIQVISNALKVWGLELILFNSPEYQRLRIDPINERSFICNYKEHWFTVRKLGKQWFNLNSLLTGPELISDTYLALFLAQLQQEGYSIFVVKGDLPDCEADQLLQMIRVQQMHRPKLIGEELAQLKEQRVHKTDLERVLEANDGSGMLDEDEEDLQRALALSRQEIDMEDEEADLRRAIQLSMQGSSRNISQDMTQTSGTNLTSEELRKRREAYFEKQQQKQQQQQQQQQQGDLSGQSSHPCERPATSSGALGSDLGDAMSEEDMLQAAVTMSLETVRNDLKTEGKKHHHHHH

No

Yes