货号：GZ8910
70 kDa mitochondrial autoantigen of primary biliary cirrhosis;anti DLAT;Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex;Dihydrolipoamide;Dihydrolipoamide S Acetyltransferase;Dihydrolipoamide S-acetyltransferase;E2 component of pyruvate dehydrogenase complex;Dihydrolipoamide S-Acetyltransferase;Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex;dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial;DLAT;DLAT;DLTA;E2;E2 component of pyruvate dehydrogenase complex;M2 antigen complex 70 kDa subunit;M2 Antigen Complex 70kD Subunit;mitochondrial;ODP2_HUMAN;PBC;PDC E2;PDC-E2;PDCE2;Pyruvate dehydrogenase complex component E2;Pyruvate dehydrogenase complex E2 subunit;S acetyltransferase component of pyruvate dehydrogenase complex.
| 别名 | 70 kDa mitochondrial autoantigen of primary biliary cirrhosis;anti DLAT;Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex;Dihydrolipoamide;Dihydrolipoamide S Acetyltransferase;Dihydrolipoamide S-acetyltransferase;E2 component of pyruvate dehydrogenase complex;Dihydrolipoamide S-Acetyltransferase;Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex;dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial;DLAT;DLAT;DLTA;E2;E2 component of pyruvate dehydrogenase complex;M2 antigen complex 70 kDa subunit;M2 Antigen Complex 70kD Subunit;mitochondrial;ODP2_HUMAN;PBC;PDC E2;PDC-E2;PDCE2;Pyruvate dehydrogenase complex component E2;Pyruvate dehydrogenase complex E2 subunit;S acetyltransferase component of pyruvate dehydrogenase complex. |
| 基因名称 | DLAT |
| 推荐应用 | |
| 反应物种 | Human,Mouse,Rat |
| 存储缓冲液 | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Human Swissprot No. | P10515 |
| Mouse Swissprot No. | Q8BMF4 |
| 特异性 | Endogenous |
| 稀释度 | IHC-P 1:500-1:2000, WB 1:2000-1:10000, IF-P/IF-F/IF-ICC 1:200-1:1000, ELISA 1:5000-1:20000, IP 1:50-1:200 |
| 参考分子量 | 69kDa |
| 预测分子量 | 69kDa |
| 运输及保存条件 | Biological ice bag transportation. Store at -20°C for at least 12 months(Do not lower than -25°C). Avoid freeze/thaw cycles |
| 宿主 | Rabbit |
| 同种型 | IgG,Kappa |
| 背景介绍 | 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 lact |
| 细胞定位 | Mitochondrion matrix. |
| 纯化 | Protein A |
| Clonality | Monoclonal |
| 细胞定位 | Mitochondrion matrix. |
周一至周日 8:00-18:00
