Overview
| 别名 | NADH dehydrogenase [ubiquinone] iron-sulfur protein 4; mitochondrial; Complex I-18 kDa; CI-18 kDa; Complex I-AQDQ; CI-AQDQ; NADH-ubiquinone oxidoreductase 18 kDa subunit |
| 基因名 | NDUFS4 |
| UniProt ID | O43181 |
| 反应种属 | Human,Mouse |
| 应用 | IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG2a |
| 克隆号 | 5W5-W5-B6 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 20 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | 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 | NADH:ubiquinone oxidoreductase subunit S4 (NDUFS4) is a nuclear-encoded accessory subunit of mitochondrial Complex I. While not directly catalytic, it is indispensable for the proper assembly, stability, and activity of the complex, facilitating electron transfer from NADH to ubiquinone in the respiratory chain. NDUFS4 is essential for mitochondrial oxidative phosphorylation, particularly in tissues with high energy demand like the brain and muscle. Pathogenic variants in NDUFS4 disrupt Complex I function and are a frequent cause of Leigh syndrome, a severe early-onset neurodegenerative disorder characterized by lactic acidosis, psychomotor regression, and brainstem lesions. |
检测原理