anti-PDHX antibody
CAT.NO. : ARG43461
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概述
产品描述 | Rabbit Polyclonal antibody recognizes PDHX. |
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反应物种 | Hu, Ms, Rat |
应用 | IP, WB |
宿主 | Rabbit |
克隆 | Polyclonal |
同位型 | IgG |
靶点名称 | PDHX |
抗原物种 | Human |
抗原 | Purified recombinant protein corresponding to human PDHX. |
偶联标记 | Un-conjugated |
Protein Full name | Pyruvate dehydrogenase protein X component, mitochondrial |
別名 | E3BP; OPDX; PDX1; proX; DLDBP |
应用说明
应用建议 |
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应用说明 | * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. |
属性
形式 | Liquid |
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纯化 | Affinity purified. |
缓冲液 | PBS (pH 7.3), 0.02% Sodium azide and 50% Glycerol. |
抗菌剂 | 0.02% Sodium azide |
稳定剂 | 50% Glycerol |
存放说明 | For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C or below. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use. |
注意事项 | For laboratory research only, not for drug, diagnostic or other use. |
生物信息
数据库连接 | Swiss-port # O00330 Human Pyruvate dehydrogenase protein X component, mitochondrial Swiss-port # Q8BKZ9 Mouse Pyruvate dehydrogenase protein X component, mitochondrial |
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基因名称 | PDHX |
全名 | pyruvate dehydrogenase complex, component X |
背景介绍 | 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.[provided by RefSeq, Oct 2009] |
生物功能 | Required 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. [UniProt] |
检测图片 (3)
ARG43461 anti-PDHX antibody WB image
Western blot: SW620 and HepG2 stained with ARG43461 anti-PDHX antibody at 1:1000 dilution.
ARG43461 anti-PDHX antibody WB image
Western blot: Rat kidney and Rat heart stained with ARG43461 anti-PDHX antibody at 1:1000 dilution.
ARG43461 anti-PDHX antibody WB image
Western blot: Mouse testis and Mouse brain stained with ARG43461 anti-PDHX antibody at 1:1000 dilution.
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