ubiquitin carboxyl-terminal hydrolase 14; deubiquitinating enzyme 14; ubiquitin thiolesterase 14; ubiquitin-specific-processing protease 14 (USP14, TGT)

Function

• proteasome-associated deubiquitinase which releases ubiquitin from the proteasome targeted ubiquitinated proteins
• ensures regeneration of ubiquitin at the proteasome
• reversibly associated subunit of the proteasome & a large fraction of proteasome-free protein within the cell
• required for the degradation of the chemokine receptor CXCR4, critical for CXCL12-induced cell chemotaxis
• inhibitor of endoplasmic reticulum-associated degradation (ERAD) under the non-stressed condition by inhibiting degradation of unfolded endoplasmic reticulum proteins via interaction with ERN1
• indispensable for synaptic development & function at neuromuscular junctions (NMJs)
• thiol-dependent hydrolysis of ester, thioester, amide, peptide & isopeptide bonds formed by the C-terminal Gly of ubiquitin
• associates with the 26S proteasome
• interacts with FANCC, CXCR4 & ERN1

Structure

• belongs to the peptidase C19 family, USP14/UBP6 subfamily
• contains 1 ubiquitin-like domain

Compartment

• cytoplasm. cell membrane

Genetics

• a segmental duplication of chromosome 18 involving ROCK1 & USP14 is one of the few features distinguishing the human genome from the chimpanzee genome
• USP14 is differentially expressed in human & chimpanzee cerebral cortex as well as fibroblast cell lines

More general terms

• ubiquitin C-terminal hydrolase
• phosphoprotein
• evolutionary divergent human protein

Additional terms

• ubiquitin (UBCEP2, UBB, UBC)

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=9097
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:9097
• OMIM: https://mirror.omim.org/entry/607274
• UniProt: http://www.uniprot.org/uniprot/P54578.html