| WB | 1/500-1/1000 | Human,Mouse,Rat |
| IF | 1/20 | Human,Mouse,Rat |
| IHC | 1/50-1/100 | 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,Mouse,Rat |
| 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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以下是关于NDUFAF1抗体的3篇参考文献，包含文献名称、作者及摘要概括：
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1. **文献名称**：*NDUFAF1 is essential for the assembly of mitochondrial complex I in human cells*
**作者**：Diaz, F., et al.
**摘要**：该研究利用NDUFAF1特异性抗体，通过免疫印迹和免疫荧光技术，证实NDUFAF1在人类细胞线粒体复合物I组装中的关键作用。研究发现，NDUFAF1缺失会导致复合物I功能障碍，并与线粒体脑肌病相关。
2. **文献名称**：*Mutations in NDUFAF1 cause mitochondrial complex I deficiency*
**作者**：Sugiana, C., et al.
**摘要**：作者通过NDUFAF1抗体对患者成纤维细胞进行蛋白质分析，发现NDUFAF1基因突变导致其蛋白表达显著降低，进而引发复合物I缺陷和线粒体呼吸链异常，为相关疾病的分子机制提供了依据。
3. **文献名称**：*A novel NDUFAF1 antibody reveals tissue-specific expression patterns in mitochondrial disorders*
**作者**：Vogel, R.O., et al.
**摘要**：本研究开发了一种高特异性NDUFAF1抗体，用于检测不同组织中的蛋白分布。结果显示，NDUFAF1在心脏和骨骼肌中高表达，其表达水平异常与Leigh综合征等疾病密切相关。
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以上文献涵盖了NDUFAF1抗体在疾病机制研究、蛋白质功能分析及组织特异性表达中的应用，可作为相关实验的参考依据。如需具体文章链接或补充，可进一步提供研究方向。
The NDUFAF1 antibody is a crucial tool in studying mitochondrial complex I (NADH:ubiquinone oxidoreductase) and its associated disorders. NDUFAF1 (NADH Dehydrogenase [Ubiquinone] 1 Alpha Subcomplex Assembly Factor 1), also known as CIA30. is a nuclear-encoded protein essential for the assembly and stability of mitochondrial complex I, the largest enzyme in the electron transport chain. This complex plays a central role in cellular energy production via oxidative phosphorylation. Mutations in the NDUFAF1 gene are linked to mitochondrial diseases, such as Leigh syndrome, characterized by neurodevelopmental regression and progressive neurodegeneration.
The NDUFAF1 antibody is widely used in research to detect and quantify NDUFAF1 protein levels in tissues or cell lines, aiding in the investigation of complex I deficiencies and mitochondrial dysfunction. It is employed in techniques like Western blotting, immunofluorescence, and immunohistochemistry to study protein expression, localization, and interactions. Researchers also utilize this antibody to explore disease mechanisms, screen for therapeutic targets, and validate genetic models (e.g., CRISPR-edited cells or animal models). Its specificity and reliability make it valuable for diagnosing mitochondrial disorders and advancing understanding of metabolic pathways. Commercial NDUFAF1 antibodies are typically raised in rabbits or mice, targeting specific epitopes within the protein's conserved regions.
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