anti-Pyruvate Dehydrogenase Complex, Component X Antibodies

Protein Summary

The pyruvate dehydrogenase (PDH) complex is located in the mitochondrial matrix and catalyzes the conversion of pyruvate to acetyl coenzyme A. The PDH complex thereby links glycolysis to Krebs cycle. The PDH complex contains three catalytic subunits, E1, E2, and E3, two regulatory subunits, E1 kinase and E1 phosphatase, and a non-catalytic subunit, E3 binding protein (E3BP). 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). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC eventually leads to cirrhosis and liver failure. Alternative splicing results in multiple transcript variants encoding distinct isoforms.

Gene names and symbols associated with PDHX

• pyruvate dehydrogenase complex, component X (pdhx) antibody
• pyruvate dehydrogenase complex component X (PDHX) antibody
• pyruvate dehydrogenase complex component X L homeolog (pdhx.L) antibody
• E3-binding protein (pdhX) antibody
• pyruvate dehydrogenase complex component X (pdhx) antibody
• Show all gene names
• pyruvate dehydrogenase complex, component X (Pdhx) antibody

• Ac1164 antibody
• AI481367 antibody
• DDBDRAFT_0168431 antibody
• DDBDRAFT_0230192 antibody
• DDB_0168431 antibody
• Show all synonym aliases
• DDB_0230192 antibody
• DLDBP antibody
• E3bp antibody
• MGC86218 antibody
• OPDX antibody
• PDHX antibody
• Pdx1 antibody
• proX antibody
• zgc:66110 antibody
• zgc:85977 antibody

Protein level used designations for PDHX

pyruvate dehydrogenase complex, component X , pyruvate dehydrogenase protein X component, mitochondrial-like , dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex , lipoyl-containing pyruvate dehydrogenase complex component X , pyruvate dehydrogenase complex, E3-binding protein subunit , pyruvate dehydrogenase complex, lipoyl-containing component X , pyruvate dehydrogenase protein X component, mitochondrial , dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex (E3-binding protein) (proX) , E3-binding protein , E3BP , proX , pyruvate dehydrogenase protein X component