Anti-DLATPolyclonalAntibody-一抗-抗體-蛋白與免疫
2023-10-29
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Anti-DLAT Polyclonal Antibody品牌：solarbio | 貨號：K004409P| 英文名稱 | Anti-DLAT Polyclonal Antibody |
|---|---|
| 宿主 | Rabbit |
| 別名 | DLTA;PDC-E2;PDCE2 |
| 應用 | WB IF |
| 稀釋比例 | WB 1:500-2000. IF 1:20-50. |
| 交叉反應 | Human Mouse Rat |
| 蛋白分子量 | 69kDa |
| Gene ID | 1737 |
| 保存 | Store at -20°C. Avoid freeze / thaw cycles. |
| 儲存液 | Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| 純化方法 | Affinity purification |
| 亞型 | IgG |
| 免疫原 | Recombinant protein of human DLAT |
| 性狀 | 液體 |
| Public Immunogen Range | Recombinant protein of human DLAT |
| Subcellular Locations | Mitochondrion matrix |
| Swiss Prot | P10515 |
| 克隆類型 | Polyclonal Antibody |
| 背景資料 | 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. |
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