PDHX (Pyruvate Dehydrogenase Complex Component X) is a Protein Coding gene. The PDHX gene, located on 11p13, is conserved in chimpanzee, Rhesus monkey, dog, cow, mouse, chicken, zebrafish, and frog. This gene encodes the E3 binding protein subunit; also known as component X of the pyruvate dehydrogenase complex. This protein tethers E3 dimers to the E2 core of the PDH complex. Defects in this gene are a cause of pyruvate dehydrogenase deficiency which results in neurological dysfunction and lactic acidosis in infancy and early childhood. This protein is also a minor antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). Among its related pathways are Pyruvate metabolism and Citric Acid (TCA) cycle and Signaling by GPCR.