Ubiquitin carboxyl-terminal hydrolase isozyme L1

Definition:

Ubiquitin-protein hydrolase involved both in the processing of ubiquitin precursors and of ubiquitinated proteins (Probable). 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 (By similarity). The homodimer may have ATP-independent ubiquitin ligase activity.

References:

[1]. E Leroy, et al. The ubiquitin pathway in Parkinson's disease. Nature. 1998 Oct 1;395(6701):451-2. [Content Brief]

[2]. C N Larsen, et al. Substrate binding and catalysis by ubiquitin C-terminal hydrolases: identification of two active site residues. Biochemistry. 1996 May 28;35(21):6735-44. [Content Brief]

[3]. Yichin Liu, et al. The UCH-L1 gene encodes two opposing enzymatic activities that affect alpha-synuclein degradation and Parkinson's disease susceptibility. Cell. 2002 Oct 18;111(2):209-18. [Content Brief]

[4]. Kaya Bilguvar, et al. Recessive loss of function of the neuronal ubiquitin hydrolase UCHL1 leads to early-onset progressive neurodegeneration. Proc Natl Acad Sci U S A. 2013 Feb 26;110(9):3489-94. [Content Brief]

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