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ubiquitin thiolesterase L3; ubiquitin C-terminal hydrolase L3; UCH-L3 (UCHL3)

Function: - ubiquitin-protein hydrolase involved both in processing of ubiquitin precursors & of ubiquitinated proteins - thiol protease - recognizes & hydrolyzes a peptide bond at the C-terminal Gly of either ubiquitin or NEDD8 - phosphorylated upon DNA damage, probably by ATM or ATR - thiol-dependent hydrolysis of ester, thioester, amide, peptide & isopeptide bonds formed by the C-terminal Gly of ubiquitin Structure: belongs to the peptidase C12 family Compartment: cytoplasm Expression: highly expressed in heart, skeletal muscle, & testis Pathology: - defect or alteration in the expression of enzymes catalyzing the removal of ubiquitin from ubiquitin-proteins conjugates may be a characteristic of neurodegenerative diseases such as Alzheimer disease, Parkinson disease, progressive supranuclear palsy, Pick disease & Kuf disease - identified as a tumor-specific antigen in colon cancer

Related

ubiquitin (UBCEP2, UBB, UBC)

General

ubiquitin C-terminal hydrolase; UCH; ubiquitin thiolesterase; ubiquitin-specific processing protease; deubiquitinating enzyme

Properties

SIZE: entity length = 230 aa MW = 26 kD COMPARTMENT: cytoplasm MOTIF: Ubiquitin binding 1 {40-57} Ser phosphorylation site {S75} cysteine residue {C95} Ser phosphorylation site {S130} histidine residue {H169} Ubiquitin binding 2 {178-186} MOTIF: aspartate residue {D184}

Database Correlations

OMIM 603090 UniProt P15374 Pfam PF01088 Kegg hsa:7347 ENZYME 3.4.19.12

References

UniProt :accession P15374