Protein Name palmitoyl-protein thioesterase 1
Organism Homo sapiens
Gene ID 5538
Gene Symbol


UniProt P50897 (PPT1_HUMAN)
Relationships Total Number of functionally related compound(s) : 157
Total Number of Articles : 109

palmitoyl-protein thioesterase 1

Gene Summary

The protein encoded by this gene is a small glycoprotein involved in the catabolism of lipid-modified proteins during lysosomal degradation. The encoded enzyme removes thioester-linked fatty acyl groups such as palmitate from cysteine residues. Defects in this gene are a cause of infantile neuronal ceroid lipofuscinosis 1 (CLN1, or INCL) and neuronal ceroid lipofuscinosis 4 (CLN4). Two transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Dec 2008]

  • palmitoyl-protein thioesterase 1
  • ceroid-palmitoyl-palmitoyl-protein thioesterase 1
  • palmitoyl-protein hydrolase 1
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