PDHX Antibody - C-terminal region (OAGA02723)

Catalog No: OAGA02723

Size:100 ul

Sample (30 ug of whole cell lysate)
A: A431
B: H1299
C: Hela
10% SDS PAGE
OAGA02723 diluted at 1:1000

Product Info

• Predicted Species Reactivity: Human
• Product Format: Liquid. 1XPBS, 10% Glycerol (pH7). 0.01% Thimerosal was added as a preservative.
• Clonality: Polyclonal
• Isotype: IgG
• Host: Rabbit
• Application: WB
• Reconstitution and Storage: Keep as concentrated solution. Aliquot and store at -20oC or below. Avoid multiple freeze-thaw cycles.
• Immunogen: Carrier-protein conjugated synthetic peptide encompassing a sequence within the C-terminus region of human PDHX. The exact sequence is proprietary.
• Purification: Purified by antigen-affinity chromatography.
• Concentration: 0.75mg/ml
• Application Info: Suggested starting dilutions are as follows: WB: 1:500-1:3000. Not yet tested in other applications. Optimal working dilutions should be determined experimentally by the end user.

Target Info

• Gene Symbol: PDHX
• Gene Full Name: pyruvate dehydrogenase complex, component X
• Alias Symbols: E3BP, OPDX, PDX1, proX, DLDBP, PDHXD
• NCBI Gene Id: 8050
• Description of Target: 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.
• Uniprot ID: O00330