| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 1/20-1/50 | Human,Mouse,Rat |
| IHC | IHC:1/100-1/200;IHF:1/50-1/200 | Human,Mouse,Rat |
| ICC | 1/50-1/200 | Human,Mouse,Rat |
| FCM | 1/20-1/100 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | AQDQ; CI 18; CI AQDQ; Complex I 18 kDa; NDUFS4;;NDUFS4 |
| WB Predicted band size | Calculated MW: 20 kDa ; Observed MW: 18 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 synthesized peptide derived from human NDUFS4 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol. |
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以下是关于NDUFS4抗体的3-4篇参考文献示例(注：文献为虚构示例，仅用于演示格式)：
1. **文献名称**：*NDUFS4 mutations impair mitochondrial complex I assembly and cause Leigh syndrome*
**作者**：Janssen, R.J., et al.
**摘要**：本研究通过免疫印迹(使用NDUFS4抗体)分析了Leigh综合征患者的线粒体复合物I表达，发现NDUFS4基因突变导致蛋白缺失，进而破坏复合物I稳定性，引发严重神经退行性症状。
2. **文献名称**：*Tissue-specific NDUFS4 knockout mouse model reveals mitochondrial dysfunction in skeletal muscle*
**作者**：Kruse, S.E., et al.
**摘要**：利用NDUFS4抗体进行免疫组化及Western blot，揭示了骨骼肌特异性NDUFS4敲除小鼠中线粒体呼吸链功能缺陷，为研究复合物I相关肌病提供了模型。
3. **文献名称**：*Antibody-based profiling of mitochondrial complex I subunits in pediatric cardiomyopathy*
**作者**：Hoefs, S.J., et al.
**摘要**：通过NDUFS4等复合物I亚基抗体的多重检测，发现儿童心肌病患者的线粒体蛋白表达异常，提示NDUFS4抗体可作为诊断工具用于筛查先天性代谢紊乱。
4. **文献名称**：*NDUFS4 antibody validation for flow cytometry in human fibroblasts*
**作者**：Quintana, A., et al.
**摘要**：本研究优化了NDUFS4抗体在流式细胞术中的应用，证实其可用于定量人成纤维细胞中线粒体复合物I的表达水平，为高通量筛选提供了可靠方法。
(注：实际文献需通过PubMed、Google Scholar等平台检索确认。)
The NDUFS4 antibody is a crucial tool for studying mitochondrial complex I (NADH:ubiquinone oxidoreductase), a key component of the electron transport chain (ETC) responsible for ATP production. NDUFS4 (NADH dehydrogenase [ubiquinone] iron-sulfur protein 4) is an 18 kDa nuclear-encoded subunit of complex I, essential for its assembly, stability, and enzymatic activity. Mutations in the NDUFS4 gene are linked to severe mitochondrial disorders, such as Leigh syndrome, characterized by neurodegeneration and metabolic dysfunction.
NDUFS4 antibodies are widely used in research to investigate complex I deficiencies, mitochondrial diseases, and metabolic pathways. They enable the detection of NDUFS4 protein levels via techniques like Western blotting, immunohistochemistry, and immunofluorescence, helping assess complex I integrity in tissues or cultured cells. Studies using these antibodies have revealed NDUFS4's role in oxidative phosphorylation, reactive oxygen species (ROS) regulation, and cellular energy homeostasis.
Additionally, NDUFS4 antibodies are employed in cancer research, as altered complex I activity is associated with tumor metabolism and progression. They also aid in evaluating therapeutic interventions targeting mitochondrial dysfunction. Validated antibodies with high specificity are critical, as cross-reactivity with other complex I subunits may lead to ambiguous results. Overall, NDUFS4 antibodies serve as indispensable tools for advancing our understanding of mitochondrial biology and related pathologies.
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