UCHL1 - Ubiquitin carboxyl-terminal hydrolase isozyme L1 - human protein (Function)
 
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UCHL1 »  Ubiquitin carboxyl-terminal hydrolase isozyme L1   [ EC 6.-.-.-EC 3.4.19.12 ]  (UCH-L1)
 
Protein also known as:  Neuron cytoplasmic protein 9.5.
Gene name:  UCHL1
Family name: Peptidase C12
Entry whose protein(s) existence is based on evidence at protein level
extend overview
1 159 1
GENE REF ISO

Function

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Overview 
Ubiquitin-protein hydrolase involved both in the processing of ubiquitin precursors and of ubiquitinated proteins. This enzyme is a thiol protease that recognizes and hydrolyzes a peptide bond at the C-terminal glycine of ubiquitin. Also binds to free monoubiquitin and may prevent its degradation in lysosomes. The homodimer may have ATP-independent ubiquitin ligase activity.  
3
  • UniProtKB
GO molecular function 
Alpha-2A adrenergic receptor bindingdefinition[GO:0031694]  
1
  • BHF-UCL
Cysteine-type endopeptidase activitydefinition[GO:0004197]  
1
  • UniProtKB
Ligase activitydefinition[GO:0016874]  
1
  • UniProtKB KW
Omega peptidase activitydefinition[GO:0008242]  
1
  • UniProtKB
Protein bindingdefinition[GO:0005515]  
2
  • IntAct
Ubiquitin bindingdefinition[GO:0043130]  
1
  • UniProtKB
Ubiquitin protein ligase bindingdefinition[GO:0031625]  
1
  • ParkinsonsUK-UCL
Ubiquitin-specific protease activitydefinition[GO:0004843] silver  
1
  • InterPro 2 GO
GO biological process 
Adult walking behaviordefinition[GO:0007628] silver  
1
  • Ortholog Compara
Axon target recognitiondefinition[GO:0007412] silver  
1
  • Ortholog Compara
Axon transport of mitochondriondefinition[GO:0019896] silver  
1
  • Ortholog Compara
Cell proliferationdefinition[GO:0008283] silver  
1
  • Ortholog Compara
Eating behaviordefinition[GO:0042755] silver  
1
  • Ortholog Compara
Muscle fiber developmentdefinition[GO:0048747] silver  
1
  • Ortholog Compara
Negative regulation of MAP kinase activitydefinition[GO:0043407]  
1
  • BHF-UCL
Neuromuscular processdefinition[GO:0050905] silver  
1
  • Ortholog Compara
Proteasome-mediated ubiquitin-dependent protein catabolic processdefinition[GO:0043161]  
1
  • ParkinsonsUK-UCL
Protein deubiquitinationdefinition[GO:0016579]  
1
  • UniProtKB
Response to ischemiadefinition[GO:0002931] silver  
1
  • Ortholog Compara
Sensory perception of paindefinition[GO:0019233] silver  
1
  • Ortholog Compara
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reaction
EC 3.4.19.12: 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).  
2
  • UniProtKB
 
More information is available from:
Pathways 
According to KEGG, this protein belongs to the following pathway:
Parkinson's disease  hsa05012+7345  
Note 
Oxidation of Met-1, Met-6, Met-12, Met-124 and Met-179 to methionine sulfoxide, and oxidation of Cys-220 to cysteine sulfonic acid have been observed in brains from Alzheimer disease (AD) and Parkinson disease (PD) patients. In AD, UCHL1 was found to be associated with neurofibrillary tangles. In contrast to UCHL3, does not hydrolyze a peptide bond at the C-terminal glycine of NEDD8.  
1
  • UniProtKB
Caution 
PubMed:9774100 reports the association of mutation Ile93Met with Parkinson disease. However, according to PubMed:16450370 this association is uncertain and UCHL1 is not a susceptibility gene for Parkinson disease.  
1
  • UniProtKB
The oxidation forms of Met-1, Met-6, Met-12, Met-124, Met-179 and Cys-220 are subject of controversy and could be the artifactual results of sample handling.  
2
  • UniProtKB
 

Biophysicochemical properties

Kinetic parameters
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

Keywords

Biological process 
Ubl conjugation pathway  definition   [KW-0833]
Molecular function 
Hydrolase  definition   [KW-0378]
Ligase  definition   [KW-0436]
Protease  definition   [KW-0645]
Thiol protease  definition   [KW-0788]
Technical term 
Reference proteome  definition   [KW-1185]
 

Further external links

Enzyme and pathway databases
SABIO-RK: P09936
Other
GeneWiki: Ubiquitin_carboxy-terminal_hydrolase_L1
GenomeRNAi: 7345
PRO: PR:P09936