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Literature summary for 1.3.8.8 extracted from

  • Gobin-Limballe, S.; Djouadi, F.; Aubey, F.; Olpin, S.; Andresen, B.S.; Yamaguchi, S.; Mandel, H.; Fukao, T.; Ruiter, J.P.; Wanders, R.J.; McAndrew, R.; Kim, J.J.; Bastin, J.
    Genetic basis for correction of very-long-chain acyl-coenzyme A dehydrogenase deficiency by bezafibrate in patient fibroblasts: toward a genotype-based therapy (2007), Am. J. Hum. Genet., 81, 1133-1143.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 1.3.8.8

Activating Compound

Activating Compound Comment Organism Structure
bezafibrate increased VLCAD residual enzyme activity in the presence of 0.4 mM bezafibrate Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
-
 Homo sapiens 5739
-

Organism

Organism UniProt Comment Textmining
Homo sapiens P49748
-
-

Source Tissue

Source Tissue Comment Organism Textmining
fibroblast
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
acyl-CoA + acceptor
-
 Homo sapiens 2,3-dehydroacyl-CoA + reduced acceptor
-
 ?
palmitoyl-CoA + acceptor
-
 Homo sapiens 2,3-unsaturated-palmitoyl-CoA + reduced acceptor
-
 ?

Synonyms

Synonyms Comment Organism
very-long-chain acyl-coenzyme A dehydrogenase
-
 Homo sapiens
VLCAD
-
 Homo sapiens