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Literature summary for 3.1.2.22 extracted from

  • Meyer, M.; Kovacs, A.D.; Pearce, D.A.
    Decreased sensitivity of palmitoyl protein thioesterase 1-deficient neurons to chemical anoxia (2017), Metab. Brain Dis., 32, 275-279 .
    View publication on PubMedView publication on EuropePMC

Localization

Localization Comment Organism GeneOntology No. Textmining
lysosome
-
Mus musculus 5764
-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
palmitoyl-[protein] + H2O Mus musculus
-
palmitate + protein
-
?

Organism

Organism UniProt Comment Textmining
Mus musculus Q88531
-
-

Source Tissue

Source Tissue Comment Organism Textmining
cerebellar granule cell neuron Mus musculus
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4-methylumbelliferyl 6-S-palmitoyl-6-thio-beta-D-glucoside + H2O
-
Mus musculus palmitate + 4-methylumbelliferyl 6-thio-beta-D-glucoside
-
?
palmitoyl-[protein] + H2O
-
Mus musculus palmitate + protein
-
?

Synonyms

Synonyms Comment Organism
CLN1
-
Mus musculus
palmitoyl protein thioesterase 1
-
Mus musculus
PPT1
-
Mus musculus

General Information

General Information Comment Organism
malfunction enzyme deficiency causes alterations in the mitochondrial respiratory chain. Enzyme-deficient neurons are more sensitive to mitochondrial complex I inhibition by 1-methyl-4-phenylpyridinium and have significantly decreased sensitivity to chemical anoxia induced by the mitochondrial complex IV inhibitor sodium azide Mus musculus