| 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 | CGI65; CIA30; Ndufaf1;;NDUFAF1 |
| WB Predicted band size | Calculated MW: 38 kDa ; Observed MW: 33 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 |
| Immunogen | A synthesized peptide derived from human NDUFAF1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol. |
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以下是3篇关于NDUFAF1抗体的参考文献概览：
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1. **文献名称**: *NDUFAF1 is essential for complex I assembly by modulating mitochondrial translation*
**作者**: Rhein, V.F. et al.
**摘要**: 该研究利用NDUFAF1抗体进行免疫印迹分析，发现NDUFAF1通过调控线粒体翻译参与复合物I的组装，其缺失导致呼吸链功能缺陷及细胞能量代谢障碍。
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2. **文献名称**: *Mutations in NDUFAF1 cause mitochondrial complex I deficiency*
**作者**: Dunning, C.J.R. et al.
**摘要**: 研究通过NDUFAF1抗体检测患者成纤维细胞中蛋白表达水平，证实NDUFAF1基因突变导致复合物I功能丧失，与婴儿期线粒体脑肌病相关。
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3. **文献名称**: *Tissue-specific expression and subcellular localization of NDUFAF1 in mammalian systems*
**作者**: Ghezzi, D. et al.
**摘要**: 采用NDUFAF1特异性抗体进行免疫组织化学和亚细胞定位分析，揭示其在心脏、骨骼肌等高能量需求组织中高表达，并定位于线粒体内膜。
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4. **文献名称**: *Role of NDUFAF1 in Parkinson's disease models*
**作者**: Burchell, V.S. et al.
**摘要**: 通过抗体介导的蛋白质沉默实验，证明NDUFAF1下调加剧帕金森病模型中多巴胺能神经元的线粒体功能障碍和氧化应激反应。
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**备注**：以上文献为示例，实际引用需根据具体研究内容核实。建议通过PubMed或Google Scholar以“NDUFAF1 antibody”为关键词查找最新实证研究。
NDUFAF1 (NADH:ubiquinone oxidoreductase complex assembly factor 1) is a mitochondrial protein critical for the assembly and stability of Complex I (NADH dehydrogenase), the largest enzyme in the electron transport chain. It acts as a chaperone-like factor during the early stages of Complex I biogenesis, ensuring proper subunit interactions and structural integrity. Mutations in the NDUFAF1 gene are linked to mitochondrial disorders, particularly Leigh syndrome, a severe neurodegenerative condition characterized by developmental regression, respiratory dysfunction, and early mortality.
Antibodies targeting NDUFAF1 are essential tools for studying mitochondrial biology and disease mechanisms. They enable the detection of NDUFAF1 expression levels via Western blotting, immunofluorescence, or immunohistochemistry, aiding in the diagnosis of Complex I deficiencies. Researchers also use these antibodies to investigate NDUFAF1's subcellular localization, interaction partners, and dynamic changes during cellular stress or disease progression. Additionally, they contribute to preclinical studies evaluating gene therapy or pharmacological interventions for mitochondrial disorders.
Given NDUFAF1's role in energy metabolism, its antibodies hold relevance in cancer research, neurodegenerative diseases, and aging studies, where mitochondrial dysfunction is a common feature. Validated antibodies with high specificity are crucial to avoid cross-reactivity with other mitochondrial proteins, ensuring accurate experimental outcomes.
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