ID:UBP13_HUMAN DESCRIPTION: RecName: Full=Ubiquitin carboxyl-terminal hydrolase 13; EC=3.4.19.12; AltName: Full=Deubiquitinating enzyme 13; AltName: Full=Isopeptidase T-3; Short=ISOT-3; AltName: Full=Ubiquitin thioesterase 13; AltName: Full=Ubiquitin-specific-processing protease 13; 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. CATALYTIC ACTIVITY: 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). ENZYME REGULATION: Specifically inhibited by spautin-1 (specific and potent autophagy inhibitor-1), a derivative of MBCQ that binds to USP13 and inhibits deubiquitinase activity. Regulated by PIK3C3/VPS34-containing complexes. The weak deubiquitinase activity in vitro suggests the existence of some mechanism that activates the enzyme. SUBUNIT: Interacts with UFD1. Interacts (via UBA domains) with SIAH2 (when ubiquitinated). TISSUE SPECIFICITY: Highly expressed in ovary and testes. DOMAIN: The UBP-type zinc finger has lost its ability to bind ubiquitin and USP13 is not activated by unanchored ubiquitin. Swapping with the UBP-type zinc finger from USP5 restores ability to bind unanchored ubiquitin and subsequent activation of the protein (PubMed:22216260). DOMAIN: The UBA domains mediate binding to ubiquitin (PubMed:22216260). SIMILARITY: Belongs to the peptidase C19 family. SIMILARITY: Contains 2 UBA domains. SIMILARITY: Contains 1 UBP-type zinc finger.
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on Q92995
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Gene Ontology (GO) Annotations with Structured Vocabulary
Molecular Function: GO:0004197 cysteine-type endopeptidase activity GO:0004843 thiol-dependent ubiquitin-specific protease activity GO:0005515 protein binding GO:0008233 peptidase activity GO:0008234 cysteine-type peptidase activity GO:0008270 zinc ion binding GO:0016787 hydrolase activity GO:0031625 ubiquitin protein ligase binding GO:0036459 thiol-dependent ubiquitinyl hydrolase activity GO:0043130 ubiquitin binding GO:0044389 ubiquitin-like protein ligase binding GO:0046872 metal ion binding GO:0051087 chaperone binding GO:0070628 proteasome binding GO:1904288 BAT3 complex binding GO:1904454 ubiquitin-specific protease activity involved in positive regulation of ERAD pathway
Biological Process: GO:0006355 regulation of transcription, DNA-templated GO:0006508 proteolysis GO:0006511 ubiquitin-dependent protein catabolic process GO:0006914 autophagy GO:0008283 cell proliferation GO:0010506 regulation of autophagy GO:0016579 protein deubiquitination GO:0030318 melanocyte differentiation GO:0035523 protein K29-linked deubiquitination GO:0044313 protein K6-linked deubiquitination GO:0050821 protein stabilization GO:0070536 protein K63-linked deubiquitination GO:1904294 positive regulation of ERAD pathway GO:1904378 maintenance of unfolded protein involved in ERAD pathway
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Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
