Literature summary for 3.1.2.22 extracted from

Lyly, A.; von Schantz, C.; Salonen, T.; Kopra, O.; Saarela, J.; Jauhiainen, M.; Kyttaelae, A.; Jalanko, A.
Glycosylation, transport, and complex formation of palmitoyl protein thioesterase 1 (PPT1) - distinct characteristics in neurons (2007), BMC Cell Biol., 8, 22.

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Protein Variants

Protein Variants	Comment	Organism
S199A	mutation shows 1-5% of normal activity	Homo sapiens
S199A/S214A	mutant enzyme with very low enzyme activity	Homo sapiens
S214A	mutant shows 31% of normal activity	Homo sapiens
S234A	mutation shows 1-5% of normal activity. Partial localization to lysosomes, although a major proportion is retained in the endoplasmic reticulum	Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates	Organism	Comment (Nat. Sub.)	Natural Products	Comment (Nat. Pro.)	Rev.	Reac.
additional information	Homo sapiens	the most severe form of neuronal ceroid lipofuscinoses, infantile neuronal ceroid lipofuscinosis (INCL), is caused by mutations in the CLN1 gene, resulting in a deficiency of the lysosomal enzyme, palmitoyl protein thioesterase 1	?	-	?

Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	P50897	precursor	-

Posttranslational Modification

Posttranslational Modification	Comment	Organism
glycoprotein	N-glycosylation of N197 and N232, but not N212, is essential for enzyme activity and intracellular transport. Deglycosylation of overexpressed PPT1 produced in neurons and fibroblasts demonstrates differentially modified PPT1 in different cell types	Homo sapiens

Substrates and Products (Substrate)

Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.	Reac.
additional information	the most severe form of neuronal ceroid lipofuscinoses, infantile neuronal ceroid lipofuscinosis (INCL), is caused by mutations in the CLN1 gene, resulting in a deficiency of the lysosomal enzyme, palmitoyl protein thioesterase 1	Homo sapiens	?	-	?

Subunits

Subunits	Comment	Organism
More	PPT1 forms oligomers	Homo sapiens

Synonyms

Synonyms	Comment	Organism
palmitoyl protein thioesterase 1	-	Homo sapiens
PPT1	-	Homo sapiens

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Release 2023.1 (January 2023)