Anti-PDHXPolyclonalAntibody-一抗-抗体-蛋白与免疫-耗材-金山科研平台 Bio-Rad伯乐官网 |伯乐授权代理 |伯乐ddPCR| 伯乐Aminex| 伯乐层析填料色谱柱

Anti-PDHXPolyclonalAntibody-一抗-抗体-蛋白与免疫

2023-10-29

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Anti-PDHX Polyclonal Antibody品牌：solarbio | 货号：K004518P

英文名称	Anti-PDHX Polyclonal Antibody
宿主	Rabbit
别名	DLDBP;E3BP;OPDX;PDX1;proX
应用	WB IHC IF
稀释比例	WB 1:500-2000. IHC 1:50-200. IF 1:10-100.
交叉反应	Human Mouse Rat
蛋白分子量	54kDa
Gene ID	8050
保存	Store at -20°C. Avoid freeze / thaw cycles.
储存液	Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
纯化方法	Affinity purification
亚型	IgG
免疫原	Recombinant protein of human PDHX
性状	液体
Public Immunogen Range	Recombinant protein of human PDHX
Subcellular Locations	Mitochondrion matrix
Swiss Prot	O00330
克隆类型	Polyclonal Antibody
背景资料	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.

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