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PPT1

Protein-coding gene in the species Homo sapiens

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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.

01Function

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.

02Clinical significance

Defects in this gene are a cause of neuronal ceroid lipofuscinosis type 1 (CLN1).

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Sources and credits

This article is adapted from the Wikipedia article PPT1, written by its contributors and licensed under CC BY-SA 4.0. Fathomly has changed the layout, removed citation markers, navigation and maintenance notices, and adjusted punctuation. This adapted version is shared under the same license. For references, see the original article.

Images, from Wikimedia Commons:

  • PDB 1eh5 EBI.jpg by Jawahar Swaminathan and MSD staff at the European Bioinformatics Institute, Public domain

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