Any feedback?
Please rate this page
(literature.php)
(0/150)

BRENDA support

Literature summary for 1.3.8.8 extracted from

  • Korematsu, S.; Kosugi, Y.; Kumamoto, T.; Yamaguchi, S.; Izumi, T.
    Novel mutation of early, perinatal-onset, myopathic-type very-long-chain acyl-CoA dehydrogenase deficiency (2009), Pediatr. Neurol., 41, 151-153.
    View publication on PubMed
Search for more literature for 1.3.8.8

Protein Variants

Protein Variants Comment Organism
997insT frameshift, heterozygote mutation of exon 10, mutation of a myopathic-type very-long-chain acyl-CoA dehydrogenase deficiency Homo sapiens
K264E A790G, heterozygote mutation of exon 9 Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
0.00087
-
 palmitoyl-CoA acyl-CoA dehydrogenase activity skin fibroblasts, lab data of a 6-year old male patient with VLCAD deficiency during rhabdomyolysis Homo sapiens
0.00471
-
 octanoyl-CoA acyl-CoA dehydrogenase activity skin fibroblasts, lab data of a 6-year old male patient with VLCAD deficiency during rhabdomyolysis Homo sapiens

Localization

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

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
skin fibroblast
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
octanoyl-CoA + acceptor
-
 Homo sapiens ?
-
 ?
palmitoyl-CoA + acceptor
-
 Homo sapiens ?
-
 ?

Synonyms

Synonyms Comment Organism
very-long-chain acyl-CoA dehydrogenase
-
 Homo sapiens