UniProt Accession

 ATX3_MOUSE; Q9CVD2 

Genbank Protein ID

 AK008675; AK030842 

Genbank Nucleotide ID

 BAB25825.3; BAC27155.1 

Protein Name

 Ataxin-3 

Protein Synonyms/Alias

 Machado-Joseph disease protein 1 homolog 

Gene Name

 Atxn3 

Gene Synonyms/Alias

 Mjd 

Created Date

 July 27, 2013 

Organism

 Mus musculus (Mouse) 

NCBI Taxa ID

 10090 

Lysine Modification

Position	Peptide	Type	References
291	RREAYFEKQQQQQQE	ubiquitination	[1]
346	TMSLETAKDNLKAER	ubiquitination	[1]

Reference

 [1] Proteomic analyses reveal divergent ubiquitylation site patterns in murine tissues.
 Wagner SA, Beli P, Weinert BT, Schölz C, Kelstrup CD, Young C, Nielsen ML, Olsen JV, Brakebusch C, Choudhary C.
 Mol Cell Proteomics. 2012 Dec;11(12):1578-85. [PMID: 22790023] 

Functional Description

 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. 

Sequence Annotation

 DOMAIN 1 180 Josephin.
 REPEAT 224 243 UIM 1.
 REPEAT 244 263 UIM 2.
 REPEAT 329 348 UIM 3.
 ACT_SITE 14 14 Nucleophile.
 ACT_SITE 119 119 Proton acceptor (By similarity).
 ACT_SITE 134 134 By similarity.
 MOD_RES 219 219 Phosphoserine.
 MOD_RES 268 268 Phosphoserine.
 MOD_RES 273 273 Phosphoserine.  

Keyword

 Complete proteome; Hydrolase; Nucleus; Phosphoprotein; Protease; Reference proteome; Repeat; Thiol protease; Transcription; Transcription regulation; Ubl conjugation; Ubl conjugation pathway. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 355 AA 

Protein Sequence

Gene Ontology

 GO:0005759; C:mitochondrial matrix; IDA:MGI.
 GO:0031966; C:mitochondrial membrane; IDA:MGI.
 GO:0042405; C:nuclear inclusion body; IDA:MGI.
 GO:0008242; F:omega peptidase activity; IEA:InterPro.
 GO:0001012; F:RNA polymerase II regulatory region DNA binding; IEA:Compara.
 GO:0004221; F:ubiquitin thiolesterase activity; IDA:UniProtKB.
 GO:0004843; F:ubiquitin-specific protease activity; IDA:UniProtKB.
 GO:0030036; P:actin cytoskeleton organization; IEA:Compara.
 GO:0034605; P:cellular response to heat; IMP:MGI.
 GO:0071218; P:cellular response to misfolded protein; IMP:UniProtKB.
 GO:0035640; P:exploration behavior; IMP:MGI.
 GO:0070932; P:histone H3 deacetylation; IEA:Compara.
 GO:0045104; P:intermediate filament cytoskeleton organization; IEA:Compara.
 GO:0000226; P:microtubule cytoskeleton organization; IEA:Compara.
 GO:0006515; P:misfolded or incompletely synthesized protein catabolic process; IMP:UniProtKB.
 GO:0035520; P:monoubiquitinated protein deubiquitination; IDA:UniProtKB.
 GO:0043161; P:proteasomal ubiquitin-dependent protein catabolic process; IMP:UniProtKB.
 GO:0010810; P:regulation of cell-substrate adhesion; IMP:MGI.
 GO:0006355; P:regulation of transcription, DNA-dependent; IEA:UniProtKB-KW.
 GO:0006351; P:transcription, DNA-dependent; IEA:UniProtKB-KW. 

Interpro

 IPR006155; Josephin.
 IPR003903; Ubiquitin-int_motif. 

Pfam

 PF02099; Josephin
 PF02809; UIM 

SMART

 SM00726; UIM 

PROSITE

 PS50957; JOSEPHIN
 PS50330; UIM 

PRINTS

 PR01233; JOSEPHIN.