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.
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
新产品
历史批次信息供参考:
Familial hemiplegic migraine: linkage to chromosome 14q32 in a Spanish kindred.
Cuenca-Leon E, et al.
Neurogenetics, 10(3), 191-198 (2009)
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)
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
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
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