Anti-Pyruvate dehydrogenase (PDHX, E3BP, OPDX, PDX1, proX, DLDBP, pyruvate dehydrogenase complex, co

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. Applications: Suitable for use in ELISA, Western Blot and Immunohistochemistry. Other applications not tested. Recommended Dilution: ELISA (Peptide): 1:32,000, Western Blot: 0.1-1ug/ml ~50kDa band observed in Human Pancreas lysate (calculated MW of 54.2kDa according to NP_003468.1). In transfected HEK293 transiently expressing PDHX a band of approx. 50kDa is observed. This band is not observed in the non-transfected HEK293., Immunohistochemistry (Paraffin): 1-3ug/ml Shows cytoplasmic staining of acinar cells, islets of Langerhans and peri-pancreatic fat cells in human pancreas., Optimal dilutions to be determined by the researcher. Peptide Blocking: P9544-01-P: Pyruvate dehydrogenase, corresponding peptide, Storage and Stability: May be stored at 4°C for short-term only. For long-term storage, aliquot and store at -20°C. Aliquots are stable for at least 12 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.