LMPD Database

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LMP002407

UniProt Annotations

Entry Information
Gene Namehydroxyacyl-CoA dehydrogenase
Protein EntryHCDH_HUMAN
UniProt IDQ16836
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=3; Name=1; IsoId=Q16836-1; Sequence=Displayed; Name=2; IsoId=Q16836-2; Sequence=VSP_016551, VSP_016552; Note=Ref.2 (AAB58153) sequence is in conflict in positions: 41:L->P, 56:R->H. ; Name=3; IsoId=Q16836-3; Sequence=VSP_016552; Note=No experimental confirmation available.;
Catalytic Activity(S)-3-hydroxyacyl-CoA + NAD(+) = 3-oxoacyl-CoA + NADH.
Disease3-alpha-hydroxyacyl-CoA dehydrogenase deficiency (HADH deficiency) [MIM
DiseaseFamilial hyperinsulinemic hypoglycemia 4 (HHF4) [MIM
FunctionPlays an essential role in the mitochondrial beta- oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.
PathwayLipid metabolism; fatty acid beta-oxidation.
PtmSuccinylation at Lys-81, adjacent to a coenzyme A binding site. Desuccinylated by SIRT5 (By similarity).
SimilarityBelongs to the 3-hydroxyacyl-CoA dehydrogenase family.
Subcellular LocationMitochondrion matrix.
SubunitHomodimer. {ECO
Tissue SpecificityExpressed in liver, kidney, pancreas, heart and skeletal muscle.