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PDHX blocking peptide

Research Use Only
GTX89576-PEP
GeneTex
Product group Proteins / Signaling Molecules
Price on request
Packing Size
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Overview

  • Supplier
    GeneTex
  • Product Name
    PDHX blocking peptide
  • Delivery Days Customer
    9
  • Certification
    Research Use Only
  • Scientific Description
    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
  • Storage Instruction
    2°C to 8°C,-20°C or -80°C
  • UNSPSC
    12352202