Anti-DLAT Monoclonal Antibody
产品名称: Anti-DLAT Monoclonal Antibody
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 |