palmitoyl-protein thioesterase 1

palmitoyl-protein thioesterase 1

A small glycoprotein enzyme encoded by PPT1 on chromosome 1p32, which removes thioester-linked fatty acyl groups such as palmitate from cysteine residues.  It is absent in neuronal ceroid lipofuscinosis types 1 and 4.
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(24) Palmitoyl-protein thioesterase 1, tripeptidyl-peptidase 1, and cathepsin D are the 3 enzymes that have been identified as deficient in some individuals with NCL.