PDHX Antibodies, cDNA Clones Research Reagents

PDHX (Pyruvate Dehydrogenase Complex Component X, also known as E3BP; OPDX; PDX1; proX; DLDBP; PDHXD), located on 11p13, is a Protein Coding gene. The gene produces a 54122 Da protein composed of 501 amino acids. The PDH complex is located in the mitochondrial matrix and catalyzes the conversion of pyruvate to acetyl coenzyme A. The mammalian PDH complex differs from that in E. coli and the other mammalian alpha-keto acid dehydrogenases by the presence of a 53-kD protein called protein X. Diseases such as Pyruvate Dehydrogenase E3-Binding Protein Deficiency and Pyruvate Dehydrogenase E1-Alpha Deficiency are associated with PDHX.

PDHX Antibody (1)

    PDHX cDNA Clone (28)

    NM_003477.2

    クローニングベクター cDNA 製品

    In lentiviral vector

    NM_175094.5

    クローニングベクター cDNA 製品

    In lentiviral vector

    PDHX qPCR Primer (1)

    PDHX の背景知識

    PDHX (Pyruvate Dehydrogenase Complex Component X) is a Protein Coding gene. The PDHX gene, located on 11p13, is conserved in chimpanzee, Rhesus monkey, dog, cow, mouse, chicken, zebrafish, and frog. 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). Among its related pathways are Pyruvate metabolism and Citric Acid (TCA) cycle and Signaling by GPCR.

    PDHX の参考文献

    • Ivanov IS, et al. (2014) Founder p.Arg 446* mutation in the pdhx gene explains over half of cases with congenital lactic acidosis in roma children. Mol Genet Metab 113 (1-2): 76-83.
    • Brown RM, et al. (2006) Pyruvate dehydrogenase e3 binding protein (protein x) deficiency. Dev Med Child Neurol 48 (9): 756-760.
    • Bottone MG, et al. (2007) Enzyme-assisted photosensitization with rose bengal acetate induces structural and functional alteration of mitochondria in hela cells. Histochem Cell Biol 127 (3): 263-271.

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