Anti-PDHX Polyclonal Antibody,BR

产品属性 Product Attributes

基本信息

英文名称： Anti-PDHX Polyclonal Antibody
中文名称： 丙酮酸脱氢酶复合物X蛋白抗体
规格： 100ul ; 50ul
宿主： Rabbit
克隆类型： Polyclonal Antibody
亚型： IgG
交叉反应：： Mouse ; Rat ; Human
应用： IP ; WB
稀释比例： WB 1:500-2000. IP 0.5μg-4μg.

抗体信息

靶标： PDHX
别名： DLDBP;E3BP;OPDX;PDX1;proX
背景说明： 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.
Swiss Prot： O00330
Gene ID： 8050
亚细胞定位： Mitochondrion matrix
免疫原： Recombinant protein of human PDHX
Public Immunogen Range： 1-300aa
理论分子量： 54kDa

其它标识

纯化方法： Affinity purification
储存液： PBS with 0.02% sodium azide，50% glycerol，pH7.3.
储存条件： Store at -20℃. Avoid freeze/thaw cycles.

产品介绍 Product Introduction

使用前请离心。如有问题请及时联系我们。
Quick spin before use. If you have any questions, please contact us.

注意事项 Considerations

1.本产品仅供科研使用。请勿用于医药、临床诊断或治疗，食品及化妆品等用途。请勿存放于普通住宅区。
2.为了您的安全和健康，请穿好实验服并佩戴一次性手套和口罩操作。
3.实验结果可由多种因素影响，相关处理只限于产品本身，不涉及其他赔偿。