PPT1

Palmitoyl-protein thioesterase 1 (PPT-1), also known as palmitoyl-protein hydrolase 1, is an is_associated_with::enzyme that in humans is encoded by the PPT1 is_associated_with::gene.

Function
PPT-1 a member of the is_associated_with::palmitoyl protein thioesterase family. PPT-1 is a small is_associated_with::glycoprotein involved in the catabolism of lipid-modified proteins during lysosomal degradation. This enzyme removes is_associated_with::thioester-linked fatty acyl groups such as is_associated_with::palmitate from is_associated_with::cysteine residues.

Clinical significance
Defects in this gene are a cause of infantile is_associated_with::neuronal ceroid lipofuscinosis 1 (CLN1, or INCL) and is_associated_with::neuronal ceroid lipofuscinosis 4 (CLN4).