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Key Documents

SRP0445

Sigma-Aldrich

Ataxin3 active human

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

Synonyme(s) :

AT3, ATX3, JOS, MJD, SCA3

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About This Item

Code UNSPSC :
12352200
Nomenclature NACRES :
NA.32

Source biologique

human

Produit recombinant

expressed in E. coli

Pureté

≥59% (SDS-PAGE)

Forme

aqueous solution

Poids mol.

42 kDa

Conditionnement

pkg of 250 μg

Numéro d'accès NCBI

NM_004993

Numéro d'accès UniProt

P54252

Conditions d'expédition

dry ice

Température de stockage

−70°C

Informations sur le gène

human ... ATXN3(4287)

Description générale

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.

Actions biochimiques/physiologiques

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.

Pictogrammes

Health hazardExclamation mark
GHS08,GHS07

Mention d'avertissement

Danger

Mentions de danger

H315,H319,H360D

Classification des risques

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

Code de la classe de stockage

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

Classe de danger pour l'eau (WGK)

WGK 1

Point d'éclair (°F)

Not applicable

Point d'éclair (°C)

Not applicable

Certificats d'analyse (COA)

Recherchez un Certificats d'analyse (COA) en saisissant le numéro de lot du produit. Les numéros de lot figurent sur l'étiquette du produit après les mots "Lot" ou "Batch".

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Consulter la Bibliothèque de documents

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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