ubiquitin thiolesterase L1; ubiquitin C-terminal hydrolase L1; UCH-L1; neuron cytoplasmic protein 9.5 (UCHL1, PARK5)
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Function
- ubiquitin-protein hydrolase involved both in the processing of ubiquitin precursors & of ubiquitinated proteins
- thiol protease that recognizes & hydrolyzes a peptide bond at the C-terminal glycine of ubiquitin
- in contrast to UCHL3, does not hydrolyze a peptide bond at the C-terminal glycine of NEDD8
- binding to free monoubiquitin may prevent its degradation in lysosomes
- homodimer may have ATP-independent ubiquitin ligase activity
- thiol-dependent hydrolysis of ester, thioester, amide, peptide & isopeptide bonds formed by the C- terminal Gly of ubiquitin
- interacts with SNCA (putative)
- interacts with COPS5
- KM=122 nM for Ub-AMC
- KM=1.20 uM for ubiquitin ethyl ester
- Vmax=0.47 umol/min/mg enzyme toward Ub-AMC
- Vmax=25 umol/min/mg enzyme toward ubiquitin ethyl ester
Structure
- homodimer
- O-glycosylated (putative)
- belongs to the peptidase C12 family
Compartment
Expression
- found in neuronal cell bodies & processes throughout the neocortex (at protein level)
- expressed in neurons & cells of the neuroendocrine system & their tumors
- weakly expressed in ovary
Pathology
- oxidation of Met-1, Met-6, Met-12, Met-124 & Met-179 to methionine sulfoxide, & oxidation of Cys-220 to cysteine sulfonic acid have been observed in brains from patients with Alzheimer disease (AD) & Parkinson disease (PD)
- in AD, UCHL1 was found to be associated with neurofibrillary tangles
More general terms
Additional terms
References
- ↑ UniProt http://www.uniprot.org/uniprot/P09936.html
- ↑ GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=UCHL1
- ↑ Wikipedia; Note: ubiquitin carboxy-terminal hydrolase L1 entry http://en.wikipedia.org/wiki/ubiquitin_carboxy-terminal_hydrolase_L1