Homo sapiens Protein: USP30
Summary
InnateDB Protein IDBP-55705.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol USP30
Protein Name ubiquitin specific peptidase 30
Synonyms
Species Homo sapiens
Ensembl Protein ENSP00000257548
InnateDB Gene IDBG-55703 (USP30)
Protein Structure
UniProt Annotation
Function Deubiquitinating enzyme that acts as a key inhibitor of mitophagy by counteracting the action of parkin (PARK2): hydrolyzes ubiquitin attached by parkin on target proteins, such as RHOT1/MIRO1 and TOMM20, thereby blocking parkin's ability to drive mitophagy (PubMed:24896179). Acts as negative regulator of mitochondrial fusion by mediating deubiquitination of MFN1 and MFN2. {ECO:0000269PubMed:18287522, ECO:0000269PubMed:24896179}.
Subcellular Localization Mitochondrion outer membrane {ECO:0000269PubMed:18287522, ECO:0000269PubMed:24896179}.
Disease Associations
Tissue Specificity Expressed in skeletal muscle, pancreas, liver and kidney. {ECO:0000269PubMed:14715245}.
Comments
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 10 experimentally validated interaction(s) in this database.
Experimentally validated
Total 10 [view]
Protein-Protein 9 [view]
Protein-DNA 1 [view]
Protein-RNA 0
DNA-DNA 0
RNA-RNA 0
DNA-RNA 0
Gene Ontology

Molecular Function
Accession GO Term
GO:0004197 cysteine-type endopeptidase activity
GO:0004221 ubiquitin thiolesterase activity
GO:0004843 ubiquitin-specific protease activity
GO:0008234 cysteine-type peptidase activity
GO:0036459 ubiquitinyl hydrolase activity
Biological Process
GO:0000422 mitochondrion degradation
GO:0006511 ubiquitin-dependent protein catabolic process
GO:0008053 mitochondrial fusion
GO:0016579 protein deubiquitination
Cellular Component
GO:0005739 mitochondrion
GO:0005741 mitochondrial outer membrane
GO:0016021 integral component of membrane
Protein Structure and Domains
PDB ID
InterPro IPR001394 Peptidase C19, ubiquitin carboxyl-terminal hydrolase
IPR028889 Ubiquitin carboxyl-terminal hydrolase-like domain
PFAM PF00443
PRINTS
PIRSF
SMART
TIGRFAMs
Post-translational Modifications
Modification
Cross-References
SwissProt Q70CQ3
PhosphoSite PhosphoSite-Q70CQ3
TrEMBL S4R3D1
UniProt Splice Variant
Entrez Gene 84749
UniGene Hs.486434
RefSeq NP_116052
HUGO HGNC:20065
OMIM 612492
CCDS CCDS9123
HPRD 15633
IMGT
EMBL AC011596 AJ586136 AK027820 AK097830 BC004868 BC022094 CH471054
GenPept AAH04868 AAH22094 BAB55392 BAG53537 CAE51936 EAW97842 EAW97844