Overview
| 别名 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 2; B17.2-like; B17.2L; Mimitin; Myc-induced mitochondrial protein; MMTN; NDUFA12-like protein |
| 基因名 | NDUFAF2 |
| UniProt ID | Q8N183 |
| 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 | 9O3-N2-F5 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 19 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:1000; IHC-1:200-1:250 |
| 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 | NADH:ubiquinone oxidoreductase complex assembly factor 2 (NDUFAF2) is a mitochondrial molecular chaperone essential for the biogenesis and assembly of complex I (NADH dehydrogenase). Complex I is the first enzyme of the mitochondrial respiratory chain, responsible for transferring electrons from NADH to ubiquinone and facilitating proton translocation across the inner mitochondrial membrane. NDUFAF2 supports the proper integration of subunits into the functional complex, thereby maintaining efficient cellular respiration and ATP production. Mutations in the NDUFAF2 gene lead to mitochondrial complex I deficiency, nuclear type 10 (MC1DN10), a severe and progressive encephalopathy characterized by Leigh-like features, including hypotonia, psychomotor regression, and lactic acidosis. |
检测原理