LMPD Database

New search | Record Overview | Ontology/Pathway Information | Domain Information | UniProt Annotations | Related Proteins
LMP000701

UniProt Annotations

Entry Information
Gene Namepyruvate dehydrogenase complex, component X
Protein EntryODPX_HUMAN
UniProt IDO00330
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=3; Name=1; IsoId=O00330-1; Sequence=Displayed; Name=2; IsoId=O00330-2; Sequence=VSP_045271; Note=No experimental confirmation available.; Name=3; IsoId=O00330-3; Sequence=VSP_053817; Note=No experimental confirmation available. Gene prediction based on EST data.;
DiseasePyruvate dehydrogenase E3-binding protein deficiency (PDHXD) [MIM
FunctionRequired for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex.
SimilarityBelongs to the 2-oxoacid dehydrogenase family.
SimilarityContains 1 lipoyl-binding domain.
Subcellular LocationMitochondrion matrix.
SubunitPart of the inner core of the multimeric pyruvate dehydrogenase complex that is composed of about 48 DLAT and 12 PDHX molecules. This core binds multiple copies of pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (DLAT, E2) and lipoamide dehydrogenase (DLD, E3). {ECO