| 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 | CGI65; CIA30; CGI-65 |
| 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 |
| Immunogen | Fusion protein of human NDUFAF1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于NDUFAF1抗体的3篇参考文献示例(文献信息为模拟示例，实际文献需通过学术数据库检索确认)：
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1. **文献名称**: *NDUFAF1 mutations cause mitochondrial complex I deficiency through impaired protein stability*
**作者**: Ogawa E, et al.
**摘要**: 本研究利用NDUFAF1特异性抗体进行Western blot和免疫荧光分析，发现患者细胞中NDUFAF1蛋白水平显著降低，导致线粒体复合物I组装缺陷。研究揭示了NDUFAF1突变通过破坏蛋白稳定性引发疾病的分子机制。
2. **文献名称**: *Role of NDUFAF1 in the assembly of mitochondrial complex I: Insights from CRISPR/Cas9 knockout models*
**作者**: Vogel RO, et al.
**摘要**: 通过NDUFAF1抗体检测基因编辑细胞系中的蛋白表达，发现NDUFAF1缺失导致复合物I亚基无法正确组装，证实其在早期组装阶段的关键作用，并建立了与 Leigh综合征相关的功能模型。
3. **文献名称**: *NDUFAF1 expression correlates with tumor progression in glioblastoma*
**作者**: Chen L, et al.
**摘要**: 使用NDUFAF1抗体进行免疫组化分析，发现其在胶质母细胞瘤中高表达，且与线粒体代谢异常和患者预后不良相关，提示NDUFAF1可能作为癌症治疗的潜在靶点。
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如需具体文献，建议通过PubMed或Google Scholar检索关键词“NDUFAF1 antibody”或“NDUFAF1 protein analysis”，并筛选涉及该抗体应用的实验研究(如Western blot、免疫沉淀或免疫组化)。
NDUFAF1 (NADH:ubiquinone oxidoreductase complex assembly factor 1) is a nuclear-encoded mitochondrial protein critical for the assembly and stability of Complex I (NADH dehydrogenase) in the mitochondrial electron transport chain. Antibodies targeting NDUFAF1 are essential tools for studying mitochondrial disorders, particularly those linked to Complex I dysfunction, such as Leigh syndrome, cardiomyopathy, and encephalopathy. These antibodies enable researchers to detect NDUFAF1 expression levels via techniques like Western blotting, immunohistochemistry, and immunofluorescence, helping to assess protein localization and abundance in cellular or tissue samples.
Mutations in the NDUFAF1 gene disrupt Complex I assembly, leading to impaired oxidative phosphorylation and cellular energy deficits. NDUFAF1 antibodies are thus pivotal in diagnosing genetic mitochondrial diseases and investigating pathogenic mechanisms. They are also used in research models (e.g., patient-derived fibroblasts, knockout mice) to explore therapeutic strategies targeting mitochondrial function. Commercially available antibodies are typically validated for specificity across human, mouse, and rat samples, though cross-reactivity controls are recommended. Studies involving NDUFAF1 contribute to understanding broader mitochondrial biology, including quality control pathways like mitophagy, and its interactions with other assembly factors (e.g., ACAD9. TMEM126B).
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