Overview
| 别名 | Persulfide dioxygenase ETHE1; mitochondrial; Ethylmalonic encephalopathy protein 1; Hepatoma subtracted clone one protein; Sulfur dioxygenase ETHE1 |
| 基因名 | ETHE1 |
| UniProt ID | O95571 |
| 反应种属 | Human,Mouse |
| 应用 | WB |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 2B9-K9-X5 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 27 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:500-1:1000 |
| 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 | ETHE1 persulfide dioxygenase (ETHE1) is a mitochondrial matrix enzyme belonging to the metallo-beta-lactamase superfamily that plays a vital role in hydrogen sulfide catabolism. It catalyzes the oxygen-dependent oxidation of glutathione persulfide into persulfite and glutathione, thereby detoxifying reactive persulfides and preventing the inhibition of cytochrome c oxidase. This activity is essential for maintaining mitochondrial energy metabolism and redox homeostasis. The protein features a characteristic alpha-beta-beta-alpha fold and coordinates a non-heme iron ion within its active site. Clinically, mutations in ETHE1 are the primary cause of ethylmalonic encephalopathy, a severe infantile disorder characterized by the accumulation of hydrogen sulfide and thiosulfate, leading to metabolic acidosis, vascular dysfunction, and progressive neurological decline. |
检测原理