Pyruvate Dehydrogenase (C54G1) Rabbit mAb

• 产品编号：CST-3205S      品牌：CST       原厂货号：3205S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

Pyruvate Dehydrogenase (C54G1) Rabbit mAb用于检测内源性丙酮酸脱氢酶α1亚基总蛋白水平。单克隆抗体通过用对应于人源丙酮酸脱氢酶蛋白序列的合成肽免疫动物制备而得丙酮酸脱氢酶复合物在存在NAD+前提下催化丙酮酸和辅酶A转化成乙酰辅酶A及CO2。乙酰辅酶A进入柠檬酸循环，草酰乙酸反应生成柠檬酸。乙酰辅酶A同时也可用于脂肪酸和胆固醇和生物合成。因此，丙酮酸的氧化脱羧反应是糖酵解，柠檬酸循环和脂代谢的关键纽带。在哺乳类的细胞中，丙酮酸脱氢酶复合物位于线粒体基质中（1）。该复合物饮食三种酶：丙酮酸脱氢酶（E1），二氢硫辛酰胺乙酰转移酶（E2）和二氢硫辛酰胺脱氢酶（E3）。丙酮酸脱氢酶（E1）包括两个亚基：α和β。该酶催化丙酮酸脱去一个CO2分子。E1　α亚基的突变通常会导致先天性的乳酸中毒，神经退行疾病和早夭（2）。

1. Strumiło, S. (2005) Acta Biochim Pol 52, 759-64.
2. Stacpoole, P.W. et al. (2003) Curr Gene Ther 3, 239-45.

原厂资料：

Specificity / Sensitivity

Pyruvate Dehydrogenase (C54G1) Rabbit mAb detects endogenous levels of total pyruvate dehydrogenase α1 subunit

Source / Purification

Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to the sequence of human pyruvate dehydrogenase.

Background

The pyruvate dehydrogenase complex catalyzes the conversion of pyruvate and CoA into acetyl-CoA and CO2 in the presence of NAD+. Acetyl-CoA then goes into the citric acid cycle where it reacts with oxaloacetate to form citrate. Acetyl-CoA is also used for fatty acid and cholesterol biosynthesis. The reaction of oxidative decarboxylation of pyruvate therefore serves as a critical link between glycolysis and the citric acid cycle and lipid metabolism. In mammalian cells, the pyruvate dehydrogenase complex is located in the mitochondrial matrix (1). This complex is comprised of three enzymes: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and dihydrolipoamide dehydrogenase (E3). Pyruvate dehydrogenase (E1) consists of two subunits: α and β. This enzyme catalyzes the removal of CO2 from pyruvate. Mutations in the α subunits of pyruvate dehydrogenase (E1) lead to congenital defects that are usually associated with lactic acidosis, neurodegeneration and early death (2).

1. Strumiło, S. (2005) Acta Biochim Pol 52, 759-64.
2. Stacpoole, P.W. et al. (2003) Curr Gene Ther 3, 239-45.

注意事项：

Storage: Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/ml BSA, 50% glycerol and less than 0.02% sodium azide. Store at –20°C. Do not aliquot the antibody.