LMPD Database

LMP002384

UniProt Annotations

Entry Information
Gene Namepalmitoyl-protein thioesterase 1
Protein EntryPPT1_HUMAN
UniProt IDP50897
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=P50897-1; Sequence=Displayed; Name=2; IsoId=P50897-2; Sequence=VSP_042033; Note=No experimental confirmation available.;
Catalytic ActivityPalmitoyl-[protein] + H(2)O = palmitate + [protein].
DiseaseCeroid lipofuscinosis, neuronal, 1 (CLN1) [MIM
FunctionRemoves thioester-linked fatty acyl groups such as palmitate from modified cysteine residues in proteins or peptides during lysosomal degradation. Prefers acyl chain lengths of 14 to 18 carbons.
SimilarityBelongs to the palmitoyl-protein thioesterase family.
Subcellular LocationLysosome.
Web ResourceName=Mutations of the PPT1 gene; Note=Retina International's Scientific Newsletter; URL="http://www.retina-international.org/files/sci-news/pptmut.htm";
Web ResourceName=NCL CLN1; Note=Neural Ceroid Lipofuscinoses mutation db; URL="http://www.ucl.ac.uk/ncl/cln1.shtml";