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

More general terms

• ubiquitin C-terminal hydrolase

Additional terms

• ubiquitin (UBCEP2, UBB, UBC)

Database

• UniProt: http://www.uniprot.org/uniprot/P15374.html
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:7347
• OMIM: https://mirror.omim.org/entry/603090