LMPD Database

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LMP001594

UniProt Annotations

Entry Information
Gene Namedihydrolipoamide dehydrogenase
Protein EntryDLDH_HUMAN
UniProt IDP09622
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=3; Name=1; IsoId=P09622-1; Sequence=Displayed; Name=2; IsoId=P09622-2; Sequence=VSP_055855; Note=No experimental confirmation available.; Name=3; IsoId=P09622-3; Sequence=VSP_055856; Note=No experimental confirmation available.;
Catalytic ActivityProtein N(6)-(dihydrolipoyl)lysine + NAD(+) = protein N(6)-(lipoyl)lysine + NADH.
CofactorName=FAD; Xref=ChEBI
DiseaseDihydrolipoamide dehydrogenase deficiency (DLDD) [MIM
FunctionLipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction.
MiscellaneousThe active site is a redox-active disulfide bond.
PtmTyrosine phosphorylated.
Sequence CautionSequence=BAD92940.1; Type=Erroneous initiation; Note=Translation N-terminally shortened.; Evidence= ;
SimilarityBelongs to the class-I pyridine nucleotide-disulfide oxidoreductase family.
Subcellular LocationMitochondrion matrix.
SubunitHomodimer. Eukaryotic pyruvate dehydrogenase complexes are organized about a core consisting of the oligomeric dihydrolipoamide acetyl-transferase, around which are arranged multiple copies of pyruvate dehydrogenase, dihydrolipoamide dehydrogenase and protein X bound by non-covalent bonds.