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UCHL1 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. Monomer. Homodimer. Interacts with SNCA. Interacts with COPS5. Found in neuronal cell bodies and processes throughout the neocortex. Expressed in neurons and cells of the diffuse neuroendocrine system and their tumors. Weakly expressed in ovary. Down-regulated in brains from Parkinson disease and Alzheimer disease patients. Belongs to the peptidase C12 family. Note: This description may include information from UniProtKB.
Protein type: Cell development/differentiation; EC; Ligase; Protease; Ubiquitin conjugating system
Chromosomal Location of Human Ortholog: 4p13
Cellular Component: cell soma; cytoplasm; cytosol; endoplasmic reticulum membrane; myelin sheath; nucleoplasm; plasma membrane
Molecular Function: alpha-2A adrenergic receptor binding; cysteine-type endopeptidase activity; ligase activity; omega peptidase activity; protein binding; thiol-dependent ubiquitin-specific protease activity; ubiquitin binding; ubiquitin protein ligase binding
Biological Process: adult walking behavior; axon target recognition; axonal transport of mitochondrion; cell proliferation; eating behavior; muscle fiber development; negative regulation of MAP kinase activity; neuromuscular process; proteasomal ubiquitin-dependent protein catabolic process; protein deubiquitination; regulation of macroautophagy; sensory perception of pain
Disease: Neurodegeneration With Optic Atrophy, Childhood-onset; Parkinson Disease 5, Autosomal Dominant
Reference #:  P09936 (UniProtKB)
Alt. Names/Synonyms: Neuron cytoplasmic protein 9.5; PARK5; PGP 9.5; PGP9.5; PGP95; ubiquitin C-terminal hydrolase; ubiquitin carboxyl-terminal esterase L1; ubiquitin carboxyl-terminal esterase L1 (ubiquitin thiolesterase); Ubiquitin carboxyl-terminal hydrolase isozyme L1; Ubiquitin thioesterase L1; UCH-L1; UCHL1
Gene Symbols: UCHL1
Molecular weight: 24,824 Da
Basal Isoelectric point: 5.33  Predict pI for various phosphorylation states
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
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