Anti-BCKDHA Polyclonal Antibody
品牌：solarbio | 货号：K006055P
| 英文名称 | Anti-BCKDHA Polyclonal Antibody |
|---|---|
| 宿主 | Rabbit |
| 别名 | BCKDE1A;MSU;MSUD1;OVD1A |
| 应用 | WB |
| 稀释比例 | WB 1:500-2000. |
| 交叉反应 | Human Mouse Rat |
| 蛋白分子量 | 50kDa |
| Gene ID | 593 |
| 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 | IgG |
| 免疫原 | Recombinant protein of human BCKDHA |
| 性状 | 液体 |
| Public Immunogen Range | Recombinant protein of human BCKDHA |
| Subcellular Locations | Mitochondrion matrix |
| Swiss Prot | P12694 |
| 克隆类型 | Polyclonal Antibody |
| 背景资料 | The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene. |