| WB | 1/500-1/1000 | Human,Mouse,Rat |
| IF | 1/20 | Human,Mouse,Rat |
| IHC | 1/50-1/100 | Human,Mouse,Rat |
| ICC | 1/50-1/200 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit; mitochondrial; Complex I-75kD; CI-75kD |
| Entrez GeneID | 4719 |
| WB Predicted band size | Calculated MW: 79 kDa; Observed MW: 79 kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human,Mouse,Rat |
| Immunogen | A synthetic peptide of human Ndufs1 |
| Formulation | Purified antibody in TBS with 0.05% sodium azide,0.05%BSA and 50% glycerol. |
+ +
以下是关于NDUFS1抗体的3篇参考文献，按您的要求整理：
1. **文献名称**：*Mitochondrial complex I deficiency caused by a homozygous NDUFS1 mutation*
**作者**：van den Heuvel, L.P., et al.
**摘要**：研究报道了一例由NDUFS1基因纯合突变导致的线粒体复合物I缺陷病例。通过Western blot和免疫组化(使用NDUFS1特异性抗体)证实患者肌肉组织中复合物I亚基表达显著降低，揭示了该突变与线粒体功能障碍和神经退行性症状的关联。
2. **文献名称**：*Structural analysis of mitochondrial complex I reveals the assembly defect of a Leigh syndrome-linked mutation*
**作者**：Stroud, D.A., et al.
**摘要**：本研究利用冷冻电镜和NDUFS1抗体对线粒体复合物I的结构进行解析，发现Leigh综合征相关的NDUFS1突变(G131S)导致复合物I组装异常，抗体标记实验进一步验证了突变对蛋白质稳定性的影响。
3. **文献名称**：*NDUFS1 knockout mouse model highlights oxidative stress and inflammation in mitochondrial disorders*
**作者**：Quintana, A., et al.
**摘要**：通过构建NDUFS1条件性敲除小鼠模型，研究使用NDUFS1抗体检测脑和肝脏组织中蛋白表达缺失，发现复合物I功能丧失导致活性氧(ROS)积累和炎症反应加剧，为治疗线粒体疾病提供新靶点。
如需更多文献或具体DOI信息，可进一步补充说明。
The NDUFS1 antibody targets the NADH:ubiquinone oxidoreductase core subunit S1 (NDUFS1), a critical component of mitochondrial complex I (NADH dehydrogenase) in the electron transport chain (ETC). Complex I facilitates NADH oxidation and ubiquinone reduction, driving proton translocation to support ATP synthesis via oxidative phosphorylation (OXPHOS). NDUFS1. located in the matrix-facing hydrophilic domain of complex I, plays a structural and catalytic role in electron transfer and enzyme assembly.
NDUFS1 mutations are linked to mitochondrial disorders, such as Leigh syndrome, characterized by neurodegeneration and metabolic dysfunction. Antibodies against NDUFS1 are essential tools for studying complex I deficiencies, enabling detection of protein expression levels via Western blotting, immunofluorescence, or immunohistochemistry. They aid in diagnosing mitochondrial diseases, evaluating tissue-specific complex I impairments, and investigating cancer metabolism, as some tumors exhibit altered complex I activity.
Research using NDUFS1 antibodies has also explored its role in aging, neurodegenerative diseases (e.g., Parkinson’s), and metabolic syndromes. These antibodies help validate CRISPR/Cas9-edited models or siRNA-mediated knockdowns in mechanistic studies. Commercial NDUFS1 antibodies are typically validated in human, mouse, or rat samples, with specificity confirmed through knockout controls. Overall, they are vital for advancing insights into mitochondrial biology and pathologies linked to OXPHOS dysfunction.
×
