84-2280-79　［取扱停止］Pyruvate Dehydrogenase (PDHX, E3BP, E3-binding Protein, OPDX, PDX1, proX, DLDBP) 100ul　219178

特徴

• 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 Western Blot, Immunohistochemistry.
• Other applications not tested.
• Recommended Dilution:Western Blot: 1:500-1:2000Immunohistochemistry: 1:50-1:200Optimal dilutions to be determined by the researcher.
• Storage and Stability:May be stored at 4°C for short-term only.
• Aliquot to avoid repeated freezing and thawing.
• Store at -20°C.
• Aliquots are stable for 12 months.
• For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.