Palmitoyl-protein thioesterase 1 (PPT-1), also known as palmitoyl-protein hydrolase 1, is an enzyme that in humans is encoded by the PPT1 gene. [5] [6] [7]
PPT-1 a member of the palmitoyl protein thioesterase family. PPT-1 is a small glycoprotein involved in the catabolism of lipid-modified proteins during lysosomal degradation. This enzyme removes thioester-linked fatty acyl groups such as palmitate from cysteine residues. [5]
Defects in this gene are a cause of neuronal ceroid lipofuscinosis type 1 (CLN1). [8]
This article incorporates text from the United States National Library of Medicine, which is in the public domain.