Goat Anti-Pyruvate dehydrogenase / PDHX Antibody | LBP73408 | Leading Biology

商品分类：多克隆抗体

产品概述：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.

来源：Goat

存储溶液：0.5 mg IgG/ml in Tris saline (20mM Tris pH7.3, 150mM NaCl), 0.02% sodium azide, with 0.5% bovine serum albumin

参考文献：Enzyme-assisted photosensitization with rose Bengal acetate induces structural and functional alteration of mitochondria in HeLa cells. Bottone MG, et al. Histochem Cell Biol, 2007 Mar. PMID 17024456.Pyruvate dehydrogenase E3 binding protein (protein X) deficiency. Brown RM, et al. Dev Med Child Neurol, 2006 Sep. PMID 16904023.A novel gross deletion caused by non-homologous recombination of the PDHX gene in a patient with pyruvate dehydrogenase deficiency. Min茅 M, et al. Mol Genet Metab, 2006 Sep-Oct. PMID 16843025.PDC-E3BP is not a dominant T-cell autoantigen in primary biliary cirrhosis. McHugh A, et al. Liver Int, 2006 May. PMID 16629643.Leigh's disease due to a new mutation in the PDHX gene. Schiff M, et al. Ann Neurol, 2006 Apr. PMID 16566017.

特异性：Human

别名：Pyruvate dehydrogenase protein X component, mitochondrial, Dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex, E3-binding protein, E3BP, Lipoyl-containing pyruvate dehydrogenase complex component X, proX, PDHX, PDX1

简单描述：Goat Polyclonal Goat Anti-Pyruvate dehydrogenase / PDHX Antibody. Validated for use in WB, IHC, E.

详情：FunctionRequired for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex.Cellular_LocationMitochondrion matrix.

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