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Literature summary for 3.1.2.22 extracted from

  • Sanders, D.N.; Farias, F.H.; Johnson, G.S.; Chiang, V.; Cook, J.R.; OBrien, D.P.; Hofmann, S.L.; Lu, J.Y.; Katz, M.L.
    A mutation in canine PPT1 causes early onset neuronal ceroid lipofuscinosis in a Dachshund (2010), Mol. Genet. Metab., 100, 349-356.
    View publication on PubMedView publication on EuropePMC

Organism

Organism UniProt Comment Textmining
Canis lupus familiaris Q5JZR0 Miniature Dachshund
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Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
0.0055
-
substrate 4-methylumbelliferyl 6-deoxy-6-thiopalmitoyl-beta-D-glucopyranoside, cerebral cortex gray matter lysate, pH not specified in the publication, temperature not specified in the publication Canis lupus familiaris

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4-methylumbelliferyl 6-deoxy-6-thiopalmitoyl-beta-D-glucopyranoside + H2O
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Canis lupus familiaris 4-methylumbelliferyl 6-deoxy-6-thio-beta-D-glucopyranoside + palmitate
-
?

Synonyms

Synonyms Comment Organism
palmitoyl protein thioesterase 1
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Canis lupus familiaris
PPT1
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Canis lupus familiaris

General Information

General Information Comment Organism
malfunction neuronal ceroid lipofuscinoses-like signs caused by single nucleotide insertion in exon 8 (homozygous) Canis lupus familiaris