Overview
| 别名 | Isovaleryl-CoA dehydrogenase; mitochondrial; IVD; Butyryl-CoA dehydrogenase |
| 基因名 | IVD |
| UniProt ID | P26440 |
| 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 | 2K6-A4-N3 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 46 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 | Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial homotetrameric flavoenzyme that catalyzes the third step in the catabolism of the amino acid leucine. It functions by dehydrogenating isovaleryl-CoA to 3-methylcrotonyl-CoA, a process that involves the transfer of electrons via electron transfer flavoprotein (ETF) to the respiratory chain. The enzyme utilizes FAD as a cofactor and relies on key catalytic residues, such as Glu283 and Arg416, to facilitate proton abstraction and substrate stabilization. Clinically, deficiency of IVD leads to isovaleric acidemia (IVA), an autosomal recessive metabolic disorder. This condition results in the toxic accumulation of organic acids, leading to metabolic acidosis and potentially fatal crises in neonates. Management typically involves dietary protein restriction and glycine supplementation to mitigate the accumulation of toxic metabolites. |
检测原理