Homo sapiens Protein: USP13
Summary
InnateDB Protein IDBP-480249.4
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol USP13
Protein Name ubiquitin specific peptidase 13 (isopeptidase T-3)
Synonyms IsoT-3; ISOT3;
Species Homo sapiens
Ensembl Protein ENSP00000417146
InnateDB Gene IDBG-66443 (USP13)
Protein Structure
UniProt Annotation
Function Deubiquitinase that mediates deubiquitination of target proteins such as BECN1, MITF, SKP2 and USP10 and is involved in various processes such as autophagy and endoplasmic reticulum- associated degradation (ERAD). Component of a regulatory loop that controls autophagy and p53/TP53 levels: mediates deubiquitination of BECN1, a key regulator of autophagy, leading to stabilize the PIK3C3/VPS34-containing complexes. Also deubiquitinates USP10, an essential regulator of p53/TP53 stability. In turn, PIK3C3/VPS34- containing complexes regulate USP13 stability, suggesting the existence of a regulatory system by which PIK3C3/VPS34-containing complexes regulate p53/TP53 protein levels via USP10 and USP13. Recruited by nuclear UFD1 and mediates deubiquitination of SKP2, thereby regulating endoplasmic reticulum-associated degradation (ERAD). Mediates stabilization of SIAH2 independently of deubiquitinase activity: binds ubiquitinated SIAH2 and acts by impairing SIAH2 autoubiquitination. Has a weak deubiquitinase activity in vitro and preferentially cleaves 'Lys-63'-linked polyubiquitin chains. In contrast to USP5, it is not able to mediate unanchored polyubiquitin disassembly. Able to cleave ISG15 in vitro; however, additional experiments are required to confirm such data. {ECO:0000269PubMed:17653289, ECO:0000269PubMed:21571647, ECO:0000269PubMed:21659512, ECO:0000269PubMed:21811243, ECO:0000269PubMed:21962518, ECO:0000269PubMed:22216260}.
Subcellular Localization
Disease Associations
Tissue Specificity Highly expressed in ovary and testes.
Comments
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 40 experimentally validated interaction(s) in this database.
Experimentally validated
Total 40 [view]
Protein-Protein 40 [view]
Protein-DNA 0
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:0005515 protein binding
GO:0008242 omega peptidase activity
GO:0008270 zinc ion binding
GO:0031625 ubiquitin protein ligase binding
GO:0036459 ubiquitinyl hydrolase activity
GO:0043130 ubiquitin binding
Biological Process
GO:0006355 regulation of transcription, DNA-templated
GO:0006511 ubiquitin-dependent protein catabolic process
GO:0006914 autophagy
GO:0008283 cell proliferation
GO:0010506 regulation of autophagy
GO:0030318 melanocyte differentiation
GO:0050821 protein stabilization
GO:0070536 protein K63-linked deubiquitination
Cellular Component
Protein Structure and Domains
PDB ID
InterPro IPR000449 Ubiquitin-associated domain/translation elongation factor EF-Ts, N-terminal
IPR001394 Peptidase C19, ubiquitin carboxyl-terminal hydrolase
IPR001607 Zinc finger, UBP-type
IPR009060 UBA-like
IPR015940 Ubiquitin-associated/translation elongation factor EF1B, N-terminal, eukaryote
IPR016652 Ubiquitinyl hydrolase
IPR028889 Ubiquitin carboxyl-terminal hydrolase-like domain
PFAM PF00627
PF00443
PF02148
PRINTS
PIRSF PIRSF016308
SMART SM00290
SM00165
TIGRFAMs
Post-translational Modifications
Modification
Cross-References
SwissProt Q92995
PhosphoSite PhosphoSite-Q92995
TrEMBL
UniProt Splice Variant
Entrez Gene 8975
UniGene Hs.604276
RefSeq
HUGO HGNC:12611
OMIM 603591
CCDS
HPRD 04666
IMGT
EMBL AC007687 AC125604 AK290338 AK302404 BC016146 CH471052 U75362
GenPept AAC63405 AAH16146 BAF83027 BAG63715 EAW78383 EAW78384