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Literature summary 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

Activating Compound

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

Localization

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

Organism

EC Number Organism UniProt Comment Textmining
1.3.8.8 Homo sapiens P49748
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
1.3.8.8 fibroblast
-
 Homo sapiens
-

Substrates and Products (Substrate)

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

Synonyms

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