생물학적 소스
human
재조합
expressed in E. coli
분석
≥59% (SDS-PAGE)
형태
aqueous solution
분자량
42 kDa
포장
pkg of 250 μg
NCBI 수납 번호
UniProt 수납 번호
배송 상태
dry ice
저장 온도
−70°C
유전자 정보
human ... ATXN3(4287)
일반 설명
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.
애플리케이션
Useful for the study of enzyme kinetics, screening inhibitors, and selectivity profiling.
생화학적/생리학적 작용
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.
신호어
Danger
유해 및 위험 성명서
Hazard Classifications
Eye Irrit. 2 - Repr. 1B - Skin Irrit. 2
Storage Class Code
6.1D - Non-combustible acute toxic Cat.3 / toxic hazardous materials or hazardous materials causing chronic effects
WGK
WGK 1
Flash Point (°F)
Not applicable
Flash Point (°C)
Not applicable
시험 성적서(COA)
제품의 로트/배치 번호를 입력하여 시험 성적서(COA)을 검색하십시오. 로트 및 배치 번호는 제품 라벨에 있는 ‘로트’ 또는 ‘배치’라는 용어 뒤에서 찾을 수 있습니다.
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)
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)
Familial hemiplegic migraine: linkage to chromosome 14q32 in a Spanish kindred.
Cuenca-Leon E, et al.
Neurogenetics, 10(3), 191-198 (2009)
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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