anti-PDHX / Pyruvate dehydrogenase antibody
CAT.NO. : ARG63746
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概述
产品描述 | Goat Polyclonal antibody recognizes PDHX / Pyruvate dehydrogenase |
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反应物种 | Hu |
预测物种 | Ms, Rat, Dog |
应用 | WB |
特异性 | This antibody is expected to recognise all reported isoforms. |
宿主 | Goat |
克隆 | Polyclonal |
同位型 | IgG |
靶点名称 | PDHX / Pyruvate dehydrogenase |
抗原物种 | Human |
抗原 | C-KSFKANLENPIRLA |
偶联标记 | Un-conjugated |
別名 | Lipoyl-containing pyruvate dehydrogenase complex component X; OPDX; proX; E3-binding protein; Dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex; E3BP; PDX1; Pyruvate dehydrogenase protein X component, mitochondrial; DLDBP |
应用说明
应用建议 |
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应用说明 | WB: Recommend incubate at RT for 1h. * 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 | Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide. |
缓冲液 | Tris saline (pH 7.3), 0.02% Sodium azide and 0.5% BSA |
抗菌剂 | 0.02% Sodium azide |
稳定剂 | 0.5% BSA |
浓度 | 0.5 mg/ml |
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 |
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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] |
研究领域 | Cancer antibody; Metabolism antibody; Signaling Transduction antibody |
预测分子量 | 54 kDa |
翻译后修饰 | Delipoylated at Lys-97 by SIRT4, delipoylation decreases the PHD complex activity. |
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