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Merck
CN

SRP0445

Sigma-Aldrich

Ataxin3 active human

recombinant, expressed in E. coli, ≥59% (SDS-PAGE)

别名:

AT3, ATX3, JOS, MJD, SCA3

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关于此项目

UNSPSC代码:
12352200
NACRES:
NA.32
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生物来源

human

重组

expressed in E. coli

方案

≥59% (SDS-PAGE)

表单

aqueous solution

分子量

42 kDa

包装

pkg of 250 μg

NCBI登记号

UniProt登记号

运输

dry ice

储存温度

−70°C

基因信息

human ... ATXN3(4287)

一般描述

Ataxin3 (ATXN3) is encoded by the gene mapped to human chromosome 14q32. The encoded protein is characterized with a ubiquitin interaction motif (UIM) domain involved in binding mono and/or polyubiquitylated proteins.

应用

Useful for the study of enzyme kinetics, screening inhibitors, and selectivity profiling.

生化/生理作用

Ataxin3 (ATXN3) is a polyglutamine neurodegenerative disease protein involved in the ubiquitin-proteasome pathway. ATXN3 performs all the functions same as ubiquitin proteases such as, reducing polyubiquitylation of 125I-lysozyme by removing ubiquitin from polyubiquitin chains, cleaving a ubiquitin protease substrate and binding the specific ubiquitin protease inhibitor, ubiquitin-aldehyde. Mutation in the gene has been observed in spinocerebellar ataxia type 3 patients.

象形图

Health hazardExclamation mark

警示用语:

Danger

危险声明

危险分类

Eye Irrit. 2 - Repr. 1B - Skin Irrit. 2

储存分类代码

6.1D - Non-combustible acute toxic Cat.3 / toxic hazardous materials or hazardous materials causing chronic effects

WGK

WGK 1

闪点(°F)

Not applicable

闪点(°C)

Not applicable

法规信息

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历史批次信息供参考:

分析证书(COA)

Lot/Batch Number

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Ataxin-3 is a histone-binding protein with two independent transcriptional corepressor activities.
Li F, et al.
The Journal of Biological Chemistry, 277(47), 45004-45012 (2002)
The polyglutamine neurodegenerative protein ataxin-3 binds polyubiquitylated proteins and has ubiquitin protease activity.
Burnett B, et al.
Human Molecular Genetics, 12(23), 3195-3205 (2003)
Familial hemiplegic migraine: linkage to chromosome 14q32 in a Spanish kindred.
Cuenca-Leon E, et al.
Neurogenetics, 10(3), 191-198 (2009)
Maria do Carmo Costa et al.
PloS one, 5(7), e11728-e11728 (2010-07-30)
During myogenesis several transcription factors and regulators of protein synthesis and assembly are rapidly degraded by the ubiquitin-proteasome system (UPS). Given the potential role of the deubiquitinating enzyme (DUB) ataxin-3 in the UPS, and the high expression of the murine
Sandra Macedo-Ribeiro et al.
PloS one, 4(6), e5834-e5834 (2009-06-09)
Spinocerebellar ataxia type-3, also known as Machado-Joseph Disease (MJD), is one of many inherited neurodegenerative disorders caused by polyglutamine-encoding CAG repeat expansions in otherwise unrelated genes. Disease protein misfolding and aggregation, often within the nucleus of affected neurons, characterize polyglutamine

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