LMPD Database

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LMP000377

UniProt Annotations

Entry Information
Gene Nameacyl-CoA dehydrogenase, very long chain
Protein EntryACADV_HUMAN
UniProt IDP49748
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=3; Name=1; IsoId=P49748-1; Sequence=Displayed; Name=2; IsoId=P49748-2; Sequence=VSP_007734; Note=No experimental confirmation available.; Name=3; IsoId=P49748-3; Sequence=VSP_046031; Note=No experimental confirmation available.;
Catalytic ActivityA very-long-chain acyl-CoA + electron-transfer flavoprotein = a very-long-chain trans-2,3-dehydroacyl-CoA + reduced electron-transfer flavoprotein.
CofactorName=FAD; Xref=ChEBI
DiseaseAcyl-CoA dehydrogenase very long-chain deficiency (ACADVLD) [MIM
FunctionActive toward esters of long-chain and very long chain fatty acids such as palmitoyl-CoA, mysritoyl-CoA and stearoyl-CoA. Can accommodate substrate acyl chain lengths as long as 24 carbons, but shows little activity for substrates of less than 12 carbons.
MiscellaneousA number of straight-chain acyl-CoA dehydrogenases of different substrate specificities are present in mammalian tissues.
PathwayLipid metabolism; mitochondrial fatty acid beta- oxidation.
PtmS-nitrosylation at Cys-237 in liver improves catalytic efficiency.
SimilarityBelongs to the acyl-CoA dehydrogenase family.
Subcellular LocationMitochondrion inner membrane.
SubunitHomodimer.