Palmitoyl-protein thioesterase 1(PPT-1 for short), also known as Palmitoyl-protein hydrolase 1, belongs to the palmitoyl-protein thioesterase family. It 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 modified cysteine residues in proteins or peptides during lysosomal degradation. Defects in PPT1 are the cause of neuronal ceroid lipofuscinosis type 1.
