Overview:

UCHL3 (Ubiquitin carboxyl-terminal hydrolase isozyme L3) is a thiol protease belonging to the deubiquitinating enzymes family and catalyzes the removal of ubiquitin from polypeptides. UCHL3 takes part in the regulation of apical membrane recycling by deubiquitinating ENAC in apical compartments (1). UCHL3 was found to hydrolyze mutant ubiquitin (UBB+1), a mutation that is associated with neurodegenerative diseases (2).

Gene Aliases:

UCH-L3

Genbank Number:

NM_006002

References:

1. Butterworth, M.B. et al: The deubiquitinating enzyme UCH-L3 regulates the apical membrane recycling of the epithelial sodium channel. J Biol Chem. 282(52):37885-93, 2007.

2. Dennissen, F.J. et al: Mutant ubiquitin (UBB+1) associated with neurodegenerative disorders is hydrolyzed by ubiquitin C-terminal hydrolase L3 (UCH-L3). FEBS Letters 585:1873-3468, 2011.