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Literature summary for 1.3.8.1 extracted from

  • van Maldegem, B.T.; Wanders, R.J.; Wijburg, F.A.
    Clinical aspects of short-chain acyl-CoA dehydrogenase deficiency (2010), J. Inherit. Metab. Dis., 33, 507-511.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 1.3.8.1

Cloned(Commentary)

Cloned (Comment) Organism
SCAD DNA and amino acid sequence determination and analysis Homo sapiens

Localization

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

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
butanoyl-CoA + acceptor Homo sapiens
-
 but-2-enoyl-CoA + reduced acceptor
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
butanoyl-CoA + acceptor
-
 Homo sapiens but-2-enoyl-CoA + reduced acceptor
-
 ?

Synonyms

Synonyms Comment Organism
SCAD
-
 Homo sapiens
short-chain acyl-CoA dehydrogenase
-
 Homo sapiens

General Information

General Information Comment Organism
additional information short-chain acyl-CoA dehydrogenase deficiency, SCADD, is an autosomal recessive inborn error of mitochondrial fatty acid oxidation due to mutations in the SCAD protein. SCADD is biochemically characterized by increased C4-carnitine in plasma and ethylmalonic acid in urine, phenotype, overview Homo sapiens
physiological function SCAD catalyzes the dehydrogenation of butyryl-CoA during the first step of the short-chain fatty acid beta-oxidation spiral Homo sapiens