LMPD Database

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LMP005644

UniProt Annotations

Entry Information
Gene Nameisovaleryl-CoA dehydrogenase
Protein EntryIVD_HUMAN
UniProt IDP26440
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=P26440-1; Sequence=Displayed; Name=2; IsoId=P26440-2; Sequence=VSP_045193; Note=No experimental confirmation available.;
Catalytic ActivityIsovaleryl-CoA + electron-transfer flavoprotein = 3-methylcrotonyl-CoA + reduced electron-transfer flavoprotein.
CofactorName=FAD; Xref=ChEBI
DiseaseIsovaleric acidemia (IVA) [MIM
PathwayAmino-acid degradation; L-leucine degradation; (S)-3- hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA
Sequence CautionSequence=BAG53799.1; Type=Erroneous initiation; Note=Translation N-terminally shortened.; Evidence= ; Sequence=EAW92413.1; Type=Erroneous gene model prediction; Evidence= ; Sequence=EAW92414.1; Type=Erroneous gene model prediction; Evidence= ; Sequence=EAW92415.1; Type=Erroneous gene model prediction; Evidence= ;
SimilarityBelongs to the acyl-CoA dehydrogenase family.
Subcellular LocationMitochondrion matrix.
SubunitHomotetramer.