MGP Database

New search | Record Overview | Ontology/Pathway Information | Domain Information | UniProt Annotations | Related Proteins
MGP000019

UniProt Annotations

Entry Information
Gene Nameacyl-CoA dehydrogenase, C-4 to C-12 straight chain
Protein Entry
UniProt IDQ5HYG7
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=P11310-1; Sequence=Displayed; Name=2; IsoId=P11310-2; Sequence=VSP_038420;
Catalytic ActivityA medium-chain acyl-CoA + electron-transfer flavoprotein = a medium-chain trans-2,3-dehydroacyl-CoA + reduced electron-transfer flavoprotein.
CofactorName=FAD; Xref=ChEBI:CHEBI:57692; Evidence={ECO:0000269|PubMed:8823176};
DiseaseAcyl-CoA dehydrogenase medium-chain deficiency (ACADMD) [MIM:201450]: An inborn error of mitochondrial fatty acid beta- oxidation which causes fasting hypoglycemia, hepatic dysfunction and encephalopathy, often resulting in death in infancy. {ECO:0000269|PubMed:10767181, ECO:0000269|PubMed:11349232, ECO:0000269|PubMed:11409868, ECO:0000269|PubMed:11486912, ECO:0000269|PubMed:1671131, ECO:0000269|PubMed:1684086, ECO:0000269|PubMed:1902818, ECO:0000269|PubMed:2251268, ECO:0000269|PubMed:2393404, ECO:0000269|PubMed:2394825, ECO:0000269|PubMed:7603790, ECO:0000269|PubMed:7929823, ECO:0000269|PubMed:8198141, ECO:0000269|PubMed:9158144}. Note=The disease is caused by mutations affecting the gene represented in this entry.
FunctionThis enzyme is specific for acyl chain lengths of 4 to 16.
MiscellaneousA number of straight-chain acyl-CoA dehydrogenases of different substrate specificities are present in mammalian tissues.
MiscellaneousUtilizes the electron transfer flavoprotein (ETF) as electron acceptor that transfers the electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase).
PathwayLipid metabolism; mitochondrial fatty acid beta- oxidation.
PtmAcetylation at Lys-307 and Lys-311 in proximity of the cofactor-binding sites reduces catalytic activity (By similarity). These sites are deacetylated by SIRT3. {ECO:0000250, ECO:0000269|PubMed:19608861}.
SimilarityBelongs to the acyl-CoA dehydrogenase family. {ECO:0000305}.
Subcellular LocationMitochondrion matrix.
SubunitHomotetramer. Interacts with the heterodimeric electron transfer flavoprotein ETF. {ECO:0000269|PubMed:15159392, ECO:0000269|PubMed:15975918, ECO:0000269|PubMed:8823176}.
  logo