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MGP000020

UniProt Annotations

Entry Information
Gene Nameacyl-CoA dehydrogenase, C-2 to C-3 short chain
Protein EntryACADS_HUMAN
UniProt IDP16219
SpeciesHuman
Comments
Comment typeDescription
Catalytic ActivityButanoyl-CoA + electron-transfer flavoprotein = 2-butenoyl-CoA + reduced electron-transfer flavoprotein.
CofactorName=FAD; Xref=ChEBI:CHEBI:57692;
DiseaseAcyl-CoA dehydrogenase short-chain deficiency (ACADSD) [MIM:201470]: An inborn error of mitochondrial fatty acid beta- oxidation resulting in acute acidosis and muscle weakness in infants, and a form of lipid-storage myopathy in adults. {ECO:0000269|PubMed:11134486, ECO:0000269|PubMed:1692038, ECO:0000269|PubMed:9499414}. Note=The disease is caused by mutations affecting the gene represented in this entry.
MiscellaneousA number of straight-chain acyl-CoA dehydrogenases of different substrate specificities are present in mammalian tissues.
PathwayLipid metabolism; mitochondrial fatty acid beta- oxidation.
SimilarityBelongs to the acyl-CoA dehydrogenase family. {ECO:0000305}.
Subcellular LocationMitochondrion matrix.
SubunitHomotetramer. {ECO:0000269|Ref.7}.
Web ResourceName=Wikipedia; Note=Butyryl-CoA dehydrogenase entry; URL="http://en.wikipedia.org/wiki/Butyryl_CoA_dehydrogenase";
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