SRP0445
Ataxin3 active human
recombinant, expressed in E. coli, ≥59% (SDS-PAGE)
Synonym(s):
AT3, ATX3, JOS, MJD, SCA3
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About This Item
NACRES:
NA.32
UNSPSC Code:
12352200
Form:
aqueous solution
Assay:
≥59% (SDS-PAGE)
Biological source:
human
Recombinant:
expressed in E. coli
Mol wt:
42 kDa
biological source
human
recombinant
expressed in E. coli
assay
≥59% (SDS-PAGE)
form
aqueous solution
mol wt
42 kDa
packaging
pkg of 250 μg
NCBI accession no.
UniProt accession no.
shipped in
dry ice
storage temp.
−70°C
Gene Information
human ... ATXN3(4287)
General description
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.
Application
Useful for the study of enzyme kinetics, screening inhibitors, and selectivity profiling.
Biochem/physiol Actions
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.
signalword
Danger
hcodes
Hazard Classifications
Eye Irrit. 2 - Repr. 1B - Skin Irrit. 2
Storage Class
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
Regulatory Information
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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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