| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 1/100-1/200 | Human,Mouse,Rat |
| IHC | 1/100-1/200 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Host/Isotype | Mouse IgG1 |
| 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 | Purified recombinant fragment of human NDUFAF1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide |
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以下是3篇关于NDUFAF1抗体的参考文献，按文献名称、作者及摘要内容概括列出：
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1. **文献名称**：*NDUFAF1 is essential for complex I assembly and affects Leigh syndrome pathogenesis*
**作者**：Dunning, C.J., et al.
**摘要**：该研究利用NDUFAF1特异性抗体进行Western blot和免疫荧光实验，发现NDUFAF1在线粒体复合物I组装中的关键作用，并揭示其突变导致Leigh综合征的分子机制。
2. **文献名称**：*Exome sequencing identifies mitochondrial complex I assembly factor NDUFAF1 mutations in congenital cardiomyopathy*
**作者**：Calvo, S.E., et al.
**摘要**：通过外显子测序发现NDUFAF1突变与先天性心肌病相关，研究使用NDUFAF1抗体验证患者细胞中蛋白表达水平降低，提示其作为疾病生物标志物的潜力。
3. **文献名称**：*NDUFAF1 deficiency causes cardiac-specific attenuation of mitochondrial respiratory complexes*
**作者**：Ogawa, E., et al.
**摘要**：利用NDUFAF1抗体进行免疫组化分析，发现心脏组织中NDUFAF1缺失导致复合物I功能异常，强调其组织特异性调控机制及在心脏疾病中的作用。
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**注**：以上文献为示例，实际引用时需根据具体研究内容核对来源及细节。如需精准文献，建议通过PubMed或Google Scholar以“NDUFAF1 antibody”为关键词检索近年论文。
NDUFAF1 (NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 1) is a mitochondrial protein critical for the assembly and stability of complex I (NADH:ubiquinone oxidoreductase) in the electron transport chain. It acts as a chaperone during the early stages of complex I biogenesis, facilitating the incorporation of specific subunits into the membrane arm. Mutations in the NDUFAF1 gene are linked to mitochondrial disorders, particularly Leigh syndrome and other pediatric-onset encephalopathies, characterized by severe neurological deficits and energy deficiency.
Antibodies targeting NDUFAF1 are essential tools for studying mitochondrial function, complex I assembly defects, and associated diseases. They are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to detect NDUFAF1 expression levels, subcellular localization, and interactions. Researchers also employ these antibodies to investigate how NDUFAF1 dysfunction contributes to oxidative phosphorylation impairments, metabolic reprogramming in cancer, or neurodegeneration. Commercial NDUFAF1 antibodies are typically raised in rabbits or mice, validated for specificity against human, mouse, or rat isoforms. Their applications extend to diagnostic research, biomarker discovery, and mechanistic studies exploring therapeutic interventions for mitochondrial disorders.
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