| WB | 1/500-1/1000 | Human,Mouse,Rat |
| IF | 1/20 | Human,Mouse,Rat |
| IHC | 咨询技术 | 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 | CGI65; CIA30; Ndufaf1 |
| Entrez GeneID | 51103 |
| WB Predicted band size | Calculated MW: 38 kDa; Observed MW: 35 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 synthetic peptide of human NDUFAF1 |
| Formulation | Purified antibody in TBS with 0.05% sodium azide,0.05%BSA and 50% glycerol. |
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以下是3篇关于NDUFAF1抗体的参考文献及其摘要概括：
1. **文献名称**: *NDUFAF1 is essential for complex I assembly by acting late in the maturation pathway*
**作者**: Vogel RO, et al.
**摘要**: 该研究利用NDUFAF1特异性抗体进行Western blot和免疫沉淀实验，揭示NDUFAF1在线粒体复合物I组装后期阶段的关键作用，其缺失导致复合物I稳定性下降及细胞能量代谢障碍。
2. **文献名称**: *Mutation screening of NDUFAF1 in Leigh syndrome patients identifies novel mutations*
**作者**: Ogilvie I, et al.
**摘要**: 通过NDUFAF1抗体对患者成纤维细胞进行蛋白表达分析，发现NDUFAF1基因突变导致蛋白表达显著降低，并证实其与Leigh综合征中线粒体复合物I缺陷的直接关联。
3. **文献名称**: *Tissue-specific expression of NDUFAF1 and its role in mitochondrial dynamics*
**作者**: Zhang J, et al.
**摘要**: 采用NDUFAF1抗体进行免疫组化及亚细胞定位研究，表明该蛋白在小鼠脑和心脏中高表达，并参与调控线粒体形态和融合过程，提示其与神经退行性疾病的潜在联系。
4. **文献名称**: *Antibody-based profiling of mitochondrial complex I assembly factors*
**作者**: Kirby DM, et al.
**摘要**: 开发针对NDUFAF1等复合物I组装因子的多克隆抗体，通过免疫印迹和免疫荧光验证其在诊断复合物I相关线粒体疾病中的应用价值，为临床检测提供工具。
(注：以上文献信息为示例性概括，实际引用需以具体论文数据为准。)
NDUFAF1 (NADH:ubiquinone oxidoreductase complex assembly factor 1) is a mitochondrial protein critical for the assembly and stability of Complex I (CI), the largest enzyme in the electron transport chain. This 75 kDa chaperone-like protein facilitates the incorporation of specific subunits into CI during its multistep biogenesis. Mutations in the NDUFAF1 gene are linked to severe mitochondrial disorders, such as Leigh syndrome, cardiomyopathy, and encephalopathy, often characterized by CI deficiency, impaired energy production, and oxidative stress.
Antibodies targeting NDUFAF1 are essential tools for studying CI assembly mechanisms, protein expression levels, and mitochondrial dysfunction in research models. They enable techniques like Western blotting, immunofluorescence, and immunohistochemistry to visualize NDUFAF1 localization in mitochondria, assess its interaction with other assembly factors (e.g., ECSIT, ACAD9), and evaluate pathological changes in patient-derived cells or engineered knockout models. Commercial antibodies are typically validated for specificity across species, including human, mouse, and rat samples.
Research using NDUFAF1 antibodies has advanced understanding of CI-related diseases and potential therapeutic strategies, such as gene therapy or metabolic interventions. These tools also contribute to exploring broader roles of NDUFAF1 in cellular processes like apoptosis, ROS regulation, and mitochondrial dynamics. Proper validation via knockdown/knout controls remains crucial due to cross-reactivity risks with structurally similar proteins.
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