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> 抗原抗體、ELISA、WB > 一抗 > Polyclonal Antibodies > Goat Anti-Pyruvate dehydrogenase / PDHX Antibody品牌 |
Leading Biology | 貨號(hào) |
AMM05103G |
產(chǎn)品分類(lèi) |
Polyclonal Antibodies | 研究領(lǐng)域 |
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產(chǎn)品概述 |
We constantly strive to ensure we provide our customers with the best antibodies. As a result of this work we offer this antibody in purified format.
We are in the process of updating our datasheets. If you have any questions regarding this update, please feel free to contact our technical support team.
This product is a high quality Goat Anti-Pyruvate dehydrogenase / PDHX antibody.
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分子量 |
54122 Da
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細(xì)胞定位 |
Antigen Cellular Localization:
Mitochondrion matrix.
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宿主 |
Goat
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種屬反應(yīng)性 |
Human
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亞型 |
IgG
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通用名 |
PDX1
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基因ID |
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UniProt ID |
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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.
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總結(jié) |
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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形式 |
0.5 mg IgG/ml in Tris saline (20mM Tris pH7.3, 150mM NaCl), 0.02% sodium azide, with 0.5% bovine serum albumin
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儲(chǔ)存條件 |
Store at +4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.
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應(yīng)用 |
WB, IHC, E
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別名 |
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
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圖像 |
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說(shuō)明書(shū) |
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數(shù)量 |
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