Overview:

UCHL1 (ubiquitin carboxyl-terminal esterase L1) is a member of the peptidase C12 family and is a thiol protease that hydrolyzes the C-terminal glycine of ubiquityl esters and amides. It is expressed in the brain and neuroendocrine system and is essential for cytoplasmic protein degradation via recycling free ubiquitin. Mutations in UCHL1 are associated with Parkinson’s Disease (PD) due to its ligase and hydrolase activity, both of which are crucial for neuronal health (1). Dysfunction of the enzyme is also associated with Alzheimer’s disease as UCHL1 was found to interact with amyloid β precursor protein and regulates the production of amyloid β protein (2).

Gene Aliases:

PGP 9.5, UCH-L1, NDGOA, PGP95, SPG79, PARK5

Genbank Number:

NM_004181

References:

1. Liu, Y. et al: The UCH-L1 Gene Encodes Two Opposing Enzymatic Activities that Affect α-Synuclein Degradation and Parkinson's Disease Susceptibility. Cell 111: 209-218, 2002.

2. Zhang, M. et al: Overexpression of ubiquitin carboxyl-terminal hydrolase L1 (UCHL1) delays Alzheimer's progression in vivo. Scientific Reports, 4:7298, 2014.