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产品简介
| 英文名称 | Anti-DLAT Monoclonal Antibody |
| 别名 | DLTA;PDC-E2;PDCE2 |
| 宿主 | Mouse |
| 稀释比例 | WB 1:1000. ICC 1:300. |
| Gene ID | 1737 |
| 保存 | Store at -20°C. Avoid freeze / thaw cycles. |
| Swiss Prot | P10515 |
| 蛋白分子量 | 69kDa |
| 储存液 | Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| 亚细胞定位 | Mitochondrion matrix |
| 免疫原 | Purified recombinant human DLAT protein fragments expressed in E.coli. |
| 亚型 | IgG1 |
| 性状 | 液体 |
| 纯化方法 | Affinity purification |
| 克隆号 | 4A4-B6-C10 |
| 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 |
| 克隆类型 | Monoclonal Antibody |
| 交叉反应 | HumanMouse |
| 应用 | WBIPICC |
| 规格 | 50ul100ul |
注意：
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。
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