DLAT 单克隆抗体-抗体-抗体-生物在线
DLAT 单克隆抗体

DLAT 单克隆抗体

商家询价

产品名称: DLAT 单克隆抗体

英文名称: Anti-DLAT Monoclonal Antibody

产品编号: K000072M

产品价格: null

产品产地: 北京市通州区马驹桥

品牌商标: solarbio

更新时间: 2023-08-11T10:26:26

使用范围: WB IP ICC

北京索莱宝科技有限公司
  • 联系人 : 索莱宝-龚思雨
  • 地址 : 北京市通州区中关村科技园区通州园金桥科技产业基地景盛南四街15号85A三层
  • 邮编 : 101102
  • 所在区域 : 北京
  • 电话 : 178****1073
  • 传真 :
  • 邮箱 : 3193328036@qq.com

 

Anti-DLAT Monoclonal Antibody

品牌:solarbio | 货号:K000072M

 

宿主 Mouse
英文名称 Anti-DLAT Monoclonal Antibody
别名 DLTA;PDC-E2;PDCE2
应用 WB ICC IP
稀释比例 WB 1:1000. ICC 1:300.
蛋白分子量 69kDa
Gene ID 1737
保存 Store at -20°C. Avoid freeze / thaw cycles.
储存液 Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
纯化方法 Affinity purification
亚型 IgG1
免疫原 Purified recombinant human DLAT protein fragments expressed in E.coli.
性状 液体
Subcellular Locations Mitochondrion matrix
Swiss Prot P10515
克隆号 4A4-B6-C10
克隆类型 Monoclonal Antibody
背景资料 This gene encodes component E2 of the multi-enzyme pyruvate dehydrogenase complex (PDC). PDC resides in the inner mitochondrial membrane and catalyzes the conversion of pyruvate to acetyl coenzyme A. The protein product of this gene, dihydrolipoamide acetyltransferase, accepts acetyl groups formed by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Dihydrolipoamide acetyltransferase is the 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 enventually leads to cirrhosis and liver failure. Mutations in this gene are also a cause of pyruvate dehydrogenase E2 deficiency which causes primary lactic acidosis in infancy and early childhood