InnateDB: Systems Biology of the Innate Immune Response

Mus musculus Protein: Atxn3

Summary

InnateDB Protein

IDBP-165324.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

Atxn3

Protein Name

ataxin 3

Synonyms

2210008M02Rik; AI463012; AI647473; ataxin-3; ATX3; Mjd; MJD1; Sca3;

Species

Mus musculus

Ensembl Protein

ENSMUSP00000021606

InnateDB Gene

IDBG-165322 (Atxn3)

Protein Structure

UniProt Annotation

Function

Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates. Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins. Involved in degradation of misfolded chaperone substrates via its interaction with STUB1/CHIP: recruited to monoubiquitinated STUB1/CHIP, and restricts the length of ubiquitin chain attached to STUB1/CHIP substrates and preventing further chain extension. In response to misfolded substrate ubiquitination, mediates deubiquitination of monoubiquitinated STUB1/CHIP. Interacts with key regulators of transcription and represses transcription: acts as a histone- binding protein that regulates transcription. {ECO:0000269PubMed:21855799}.

Subcellular Localization

Nucleus {ECO:0000250}.

Disease Associations

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 13 experimentally validated interaction(s) in this database.
They are also associated with 60 interaction(s) predicted by orthology.

Experimentally validated
Total	13 [view]
Protein-Protein	13 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Predicted by orthology
Total	60 [view]

Gene Ontology

Molecular Function

Accession	GO Term
GO:0001012	RNA polymerase II regulatory region DNA binding
GO:0004221	ubiquitin thiolesterase activity
GO:0004407	histone deacetylase activity
GO:0004843	ubiquitin-specific protease activity
GO:0005515	protein binding
GO:0008242	omega peptidase activity
GO:0031625	ubiquitin protein ligase binding

Biological Process

GO:0006351	transcription, DNA-templated
GO:0006355	regulation of transcription, DNA-templated
GO:0006511	ubiquitin-dependent protein catabolic process
GO:0006515	misfolded or incompletely synthesized protein catabolic process
GO:0010810	regulation of cell-substrate adhesion
GO:0034605	cellular response to heat
GO:0035520	monoubiquitinated protein deubiquitination
GO:0035640	exploration behavior
GO:0043161	proteasome-mediated ubiquitin-dependent protein catabolic process
GO:0070932	histone H3 deacetylation
GO:0071218	cellular response to misfolded protein