Pyruvate dehydrogenase complex, E3-binding protein subunit (PDHX)

Pyruvate dehydrogenase complex, E3-binding protein subunit (PDHX) is a structural component of the mitochondrial pyruvate dehydrogenase complex, essential in cellular respiration. PDHX encodes E3 binding protein, which tethers the E3 (dihydrolipoamide dehydrogenase) enzyme to the E2 core, ensuring proper assembly and function of the PDH complex. The PDH complex catalyzes the conversion of pyruvate to acetyl-CoA, linking glycolysis to the citric acid cycle and ATP production. Mutations in PDHX can cause pyruvate dehydrogenase deficiency, leading to lactic acidosis and severe neurological problems, primarily manifesting in infancy or early childhood. PDHX is also a minor autoantigen in primary biliary cholangitis, contributing to autoimmune destruction of bile duct epithelial cells and subsequent liver dysfunction[1][2][3][6].

Other names

E3 binding proteinPyruvate dehydrogenase protein X component, mitochondrialDihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complexLipoyl-containing pyruvate dehydrogenase complex component XPyruvate dehydrogenase complex, component XPyruvate dehydrogenase complex, lipoyl-containing component XproXOPDXDLDBPPDHXD

Drugs may target the pyruvate dehydrogenase complex by activating residual enzymatic activity, reducing pyruvate accumulation, or managing lactic acidosis; not directly targeting PDHX but acting downstream or via cofactors[2].