| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/50-1/200 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/2000-1/5000 | Human,Mouse,Rat |
| Aliases | AQDQ; CI-18 |
| WB Predicted band size | 20 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 | Fusion protein of human NDUFS4 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于NDUFS4抗体的文献示例(注：以下内容为模拟生成的参考格式，实际文献需通过学术数据库查询):
1. **文献名称**: "NDUFS4 deficiency in mitochondrial complex I drives metabolic remodeling and neurodegeneration"
**作者**: Smith A, et al.
**摘要**: 本研究利用NDUFS4特异性抗体，通过Western blot和免疫组化分析NDUFS4基因敲除小鼠模型中线粒体复合物I的蛋白表达变化，揭示了NDUFS4缺失导致能量代谢异常与神经退行性病变的机制。
2. **文献名称**: "Antibody-based profiling of mitochondrial complex I subunits in Leigh syndrome patients"
**作者**: Chen L, et al.
**摘要**: 通过NDUFS4抗体对Leigh综合征患者肌肉活检样本进行免疫荧光染色，发现NDUFS4蛋白表达显著降低，提示其作为该疾病潜在生物标志物的价值。
3. **文献名称**: "Validation of a commercial NDUFS4 antibody for detecting mitochondrial dysfunction in cellular models"
**作者**: Johnson R, et al.
**摘要**: 本文系统验证了某品牌NDUFS4抗体的特异性，通过siRNA敲低实验和质谱分析确认其识别目标蛋白的能力，并应用于细胞氧化应激模型中复合物I的功能研究。
4. **文献名称**: "NDUFS4 mutations alter protein stability revealed by antibody-mediated quantification in fibroblast cultures"
**作者**: González S, et al.
**摘要**: 利用NDUFS4抗体对患者成纤维细胞进行蛋白定量，发现特定突变导致NDUFS4降解加速，为靶向蛋白稳定性的治疗策略提供依据。
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**建议**：实际文献可通过PubMed或Google Scholar以关键词“NDUFS4 antibody”、“mitochondrial complex I NDUFS4”检索，重点关注抗体验证、疾病机制或诊断应用类研究。
NDUFS4 antibody is a specialized tool used to detect the NDUFS4 protein, a critical subunit of mitochondrial Complex I (NADH:ubiquinone oxidoreductase) in the electron transport chain. Complex I, the largest multi-subunit enzyme in oxidative phosphorylation, plays a central role in ATP production by transferring electrons from NADH to ubiquinone. The NDUFS4 subunit, encoded by the nuclear *NDUFS4* gene, is essential for Complex I assembly, stability, and enzymatic activity. Mutations or deficiencies in NDUFS4 are linked to severe mitochondrial disorders, such as Leigh syndrome, characterized by progressive neurodegeneration, muscle weakness, and metabolic crises.
NDUFS4 antibodies are widely utilized in research to study mitochondrial dysfunction in diseases, including neurodegenerative conditions, cancer, and metabolic syndromes. These antibodies enable the detection of NDUFS4 protein levels via techniques like Western blotting, immunohistochemistry, or immunofluorescence, aiding in the assessment of Complex I integrity. Studies using NDUFS4 knockout models highlight its role in energy metabolism, reactive oxygen species regulation, and apoptosis. Additionally, NDUFS4 antibodies help explore therapeutic interventions targeting mitochondrial respiration or gene therapy approaches. As mitochondrial research advances, NDUFS4 remains a key biomarker for understanding cellular bioenergetics and disease mechanisms.
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