MitoProteome Database

New search | Record Overview | Ontology/Pathway Information | Domain Information | UniProt Annotations | Related Proteins

MT000008

UniProt Annotations

Entry Information

Gene Name	acyl-CoA dehydrogenase, C-2 to C-3 short chain
Protein Entry
UniProt ID
Species	Human

Comments

Comment type	Description
Catalytic Activity	Butanoyl-CoA + electron-transfer flavoprotein = 2-butenoyl-CoA + reduced electron-transfer flavoprotein.
Cofactor	Name=FAD; Xref=ChEBI:CHEBI:57692;
Disease	Acyl-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.
Miscellaneous	A number of straight-chain acyl-CoA dehydrogenases of different substrate specificities are present in mammalian tissues.
Pathway	Lipid metabolism; mitochondrial fatty acid beta- oxidation.
Similarity	Belongs to the acyl-CoA dehydrogenase family. {ECO:0000305}.
Subcellular Location	Mitochondrion matrix.
Subunit	Homotetramer. {ECO:0000269\|Ref.7}.
Web Resource	Name=Wikipedia; Note=Butyryl-CoA dehydrogenase entry; URL="http://en.wikipedia.org/wiki/Butyryl_CoA_dehydrogenase";