丙酮酸脱氢酶复合物X蛋白抗体
规格：1mg/1ml
英文名： PDHX
别名： Dihydrolipoamide dehydrogenase binding protein of pyruvate dehydrogenase complex; Dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex; DLDBP; E3 binding protein; E3-bindin
分子量： 48kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human PDHX
交叉反应：Human, Mouse, Rat, Dog,
细胞定位：
丙酮酸脱氢酶复合物X蛋白抗体产品介绍：background: PDHX is component X of the pyruvate dehydrogenase (PDH) complex. It is 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. PDHX is expressed in the mithochondrion. Function: 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. Subunit: Part of the inner core of the multimeric pyruvate dehydrogenase complex that is composed of about 48 DLAT and 12 PDHX molecules. This core binds multiple copies of 丙酮酸脱氢酶复合物X蛋白抗体pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (DLAT, E2) and lipoamide dehydrogenase (DLD, E3). Subcellular Location: Mitochondrion matrix. DISEASE: Pyruvate dehydrogenase E3-binding protein deficiency (PDHXD) [MIM:245349]: A metabolic disorder characterized by decreased activity of the pyruvate dehydrogenase complex without observable reduction in the activities 丙酮酸脱氢酶复合物X蛋白抗体of enzymes E1, E2, or E3. Clinical features include hypotonia and psychomotor retardation. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the 2-oxoacid dehydrogenase family. Contains 1 lipoyl-binding domain. Database links: UniProtKB/Swiss-Prot: O00330.3 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
丙酮酸脱氢酶复合物X蛋白抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 **学 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid