徐州威格顿生物科技有限公司
威格顿生物
Weigedun Biotechnology
NDUFS5 Mouse mAb
规格信息
| 规格 | 货号 |
|---|---|
| 50ul | WGD-Z-2611023 |
| 100ul | WGD-Z-2611023 |
详细参数
| 别名 | NADH dehydrogenase [ubiquinone] iron-sulfur protein 5; Complex I-15 kDa; CI-15 kDa; NADH-ubiquinone oxidoreductase 15 kDa subunit |
| 基因名 | NDUFS5 |
| UniProt ID | O43920 |
| 反应种属 | Human |
| 应用 | WB |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 1R1-U7-B5 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 12 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-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 | NADH:ubiquinone oxidoreductase subunit S5 (NDUFS5) is a nuclear-encoded accessory subunit of mitochondrial respiratory chain complex I. Localized to the inner mitochondrial membrane, it belongs to the iron-sulfur protein fragment and is essential for the stability and assembly of the complex, although it does not participate directly in electron transfer. The protein contains conserved cysteine motifs that form structural disulfide bonds. NDUFS5 is widely expressed and is vital for maintaining efficient respiratory electron transport. Pathogenic variants in the NDUFS5 gene are associated with mitochondrial complex I deficiency, a heterogeneous disorder that presents with encephalopathy and lactic acidosis. It is also linked to progressive leukoencephalopathy with ovarian failure, reflecting the high energy requirements of neurological and reproductive tissues. |