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Protein information

Protein name Ubiquitin carboxyl-terminal hydrolase isozyme L1 
Synonyms EC
Ubiquitin thioesterase L1
Neuron cytoplasmic protein 9.5
PGP 9.5
EC 6.-.-.-
Gene name UCHL1 .
Source Homo sapiens (Human)
Expression system COS-7
Full length  223
Precursor  no
Mature form length  223
Molecular weight (Da)  24824
Swiss-Prot Sequence Version  November 1, 1990 (Sequence version 2)
Comments  UCH-L1 is a neuronal-specific member of the ubiquitin C-terminal hydrolase (UCH) family of DUBs, and is involved in several neurodegenerative diseases and cancer. It is present in neuronal inclusions associated with Parkinsonís disease, Alzheimerís disease, and Lewy body disease.



Ubiquitylation features

Ubiquitylation site K4, K65, K71, K157 (preferred)

Ubiquitylation type Pluri-ubiquitylation
Features Monoubiquitylation restricts enzyme activity by preventing binding to ubiquitylated targets. Permanent modification, as mimicked by a ubiquitin-UCH-L1 fusion, inhibits enzyme capacity to increase free ubiquitin levels in cells. Ubiquitylation sites were identified by mass spectrometry. Ubiquitylation of other lysines and probably N-terminal ubiquitylation may also occur.

Ubiquitylation/deubiquitylation machinery

E2 Not determined
E3 Not determined
E4/AP Not determined
Comments UCH-L1 catalyzes its own deubiquitylation in an intramolecular manner, regulating the lifetime of this modification.


[1]Meray RK, Lansbury PT Jr.
Reversible monoubiquitination regulates the Parkinson's disease-associated ubiquitin hydrolase UCH-L1. J Biol Chem. 2007 Jan 26 [Epub ahead of print]


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