Overview
| 别名 | Dihydrolipoyl dehydrogenase; mitochondrial; Dihydrolipoamide dehydrogenase; Glycine cleavage system L protein |
| 基因名 | Lipoamide Dehydrogenase |
| UniProt ID | P09622 |
| 反应种属 | Human |
| 应用 | WB,IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 5T6-Y8-R6 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 54 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:1000; IHC-1:100-1:200 |
| 存储缓冲液 | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.09% sodium azide |
| 保存温度 | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| 背景信息 | Dihydrolipoamide dehydrogenase (Lipoamide Dehydrogenase) is a mitochondrial flavoprotein oxidoreductase that serves as the essential E3 component for multiple α-ketoacid dehydrogenase complexes, including pyruvate, α-ketoglutarate, and branched-chain complexes, as well as the glycine cleavage system. It catalyzes the reoxidation of dihydrolipoamide while reducing NAD+ to NADH, utilizing a conserved redox-active disulfide and FAD cofactor. Beyond its canonical metabolic roles, the enzyme exhibits diaphorase activity and contributes to cellular redox homeostasis. Clinically, biallelic mutations in the DLD gene cause dihydrolipoamide dehydrogenase deficiency, a severe mitochondrial disorder characterized by lactic acidosis, progressive neurological deterioration, and Leigh-like lesions. |
检测原理