LMPD Database

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LMP001128

UniProt Annotations

Entry Information
Gene Nameacyl-CoA dehydrogenase family, member 8
Protein EntryACAD8_HUMAN
UniProt IDQ9UKU7
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=3; Name=1; IsoId=Q9UKU7-1; Sequence=Displayed; Name=2; IsoId=Q9UKU7-2; Sequence=VSP_055780, VSP_055781; Note=No experimental confirmation available.; Name=3; IsoId=Q9UKU7-3; Sequence=VSP_055779, VSP_055782; Note=No experimental confirmation available.;
Catalytic ActivityIsobutyryl-CoA + ETF = methylacrylyl-CoA + reduced ETF.
CofactorName=FAD; Xref=ChEBI
DiseaseIsobutyryl-CoA dehydrogenase deficiency (IBDD) [MIM
FunctionHas very high activity toward isobutyryl-CoA. Is an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Plays a role in transcriptional coactivation within the ARC complex.
PathwayAmino-acid degradation; L-valine degradation.
SimilarityBelongs to the acyl-CoA dehydrogenase family.
Subcellular LocationMitochondrion .
SubunitHomotetramer, formed by a dimer of dimers. Subunit of the large multiprotein complex ARC/DRIP. {ECO
Tissue SpecificityDetected at comparable levels in all tissues examined (heart, lung, brain, skeletal muscle, pancreas and placenta). Weakly expressed in liver and kidney.