免 疫 原：KLH conjugated synthetic peptide derived from human PDHX
抗原表位：231-330/501
SWISS：O00330
Gene ID ：8050
Human Gene ID：8050

PDHX is component X of the pyruvate dehydrogenase (PDH) complex. It is required 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. PDHX is expressed in the mithochondrion.
Function：Required 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.
Subunit：Part 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 de
Subcellular Location：Mitochondrion matrix.
DISEASE：Pyruvate dehydrogenase E3-binding protein deficiency (PDHXD) [MIM:245349]: A metabolic disorder characterized by decreased activity of the pyruvate dehydrogenase complex without observable reduction in the activities of enzymes E1, E2, or E3. Clinical fea
Similarity：Belongs to the 2-oxoacid dehydrogenase family.
Contains 1 lipoyl-binding domain.
Important Note：This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.