ATXN3 - Ataxin-3 - human protein (Function)
 
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ATXN3 »  Ataxin-3   [ EC 3.4.19.12 ]
 
Protein also known as:  Spinocerebellar ataxia type 3 protein.
Gene name:  ATXN3
Entry whose protein(s) existence is based on evidence at protein level
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GENE REF ISO

Function

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Overview 
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.  
4
  • CuratedUniProtKB
GO molecular function 
ATPase bindingdefinition[GO:0051117]  
1
  • IPIParkinsonsUK-UCL
Identical protein bindingdefinition[GO:0042802]  
1
  • IPIIntAct
Lys48-specific deubiquitinase activitydefinition[GO:1990380]  
1
  • IDAParkinsonsUK-UCL
Lys63-specific deubiquitinase activitydefinition[GO:0061578]  
1
  • IDAParkinsonsUK-UCL
Omega peptidase activitydefinition[GO:0008242] silver  
  • IEAInterPro 2 GO
Protein bindingdefinition[GO:0005515]  
6
  • IPIParkinsonsUK-UCL
  • IPIIntAct
Ubiquitin protein ligase bindingdefinition[GO:0031625]  
1
  • IPIParkinsonsUK-UCL
  • ISSOrtholog Curator
Ubiquitin thiolesterase activitydefinition[GO:0004221]  
  • ISSOrtholog Curator
Ubiquitin-specific protease activitydefinition[GO:0004843]  
  • ISSOrtholog Curator
GO biological process 
Actin cytoskeleton organizationdefinition[GO:0030036]  
1
  • IMPMGI
Cell deathdefinition[GO:0008219]  
  • IEAUniProtKB KW
Cellular response to misfolded proteindefinition[GO:0071218]  
  • ISSOrtholog Curator
Intermediate filament cytoskeleton organizationdefinition[GO:0045104]  
1
  • IMPMGI
Microtubule cytoskeleton organizationdefinition[GO:0000226]  
1
  • IMPMGI
Misfolded or incompletely synthesized protein catabolic processdefinition[GO:0006515]  
  • ISSOrtholog Curator
Monoubiquitinated protein deubiquitinationdefinition[GO:0035520]  
  • ISSOrtholog Curator
Nervous system developmentdefinition[GO:0007399]  
1
  • TASPINC
Nucleotide-excision repairdefinition[GO:0006289]  
1
  • TASPINC
Proteasome-mediated ubiquitin-dependent protein catabolic processdefinition[GO:0043161]  
  • ISSOrtholog Curator
Protein K48-linked deubiquitinationdefinition[GO:0071108]  
1
  • IDAParkinsonsUK-UCL
Protein K63-linked deubiquitinationdefinition[GO:0070536]  
1
  • IDAParkinsonsUK-UCL
Regulation of cell-substrate adhesiondefinition[GO:0010810]  
1
  • IMPMGI
Regulation of transcription, DNA-templateddefinition[GO:0006355]  
  • IEAUniProtKB KW
Synaptic transmissiondefinition[GO:0007268]  
1
  • TASPINC
Transcription, DNA-templateddefinition[GO:0006351]  
  • IEAUniProtKB KW
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reaction
EC 3.4.19.12: Thiol-dependent hydrolysis of ester, thioester, amide, peptide and isopeptide bonds formed by the C-terminal Gly of ubiquitin (a 76-residue protein attached to proteins as an intracellular targeting signal).  
1
  • CuratedUniProtKB
 
More information is available from:
Pathways 
According to KEGG, this protein belongs to the following pathway:
Protein processing in endoplasmic reticulum  hsa04141+4287  
 

Keywords

Biological process 
Transcription  definition   [KW-0804]
Transcription regulation  definition   [KW-0805]
Ubl conjugation pathway  definition   [KW-0833]
Molecular function 
Hydrolase  definition   [KW-0378]
Protease  definition   [KW-0645]
Thiol protease  definition   [KW-0788]
Technical term 
Reference proteome  definition   [KW-1185]
 

Further external links

GeneWiki: Ataxin_3
GenomeRNAi: 4287
PRO: PR:P54252