| 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 | My013; C6orf66; HRPAP20; HSPC125; bA22L21.1 |
| Entrez GeneID | 29078 |
| WB Predicted band size | Calculated MW: 20 kDa; Observed MW: 20 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 | Recombinant protein of human NDUFAF4 |
| Formulation | Purified antibody in TBS with 0.05% sodium azide,0.05%BSA and 50% glycerol. |
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以下是3篇与NDUFAF4抗体相关的代表性文献(注：部分文献为假设性示例，实际研究需通过PubMed/Google Scholar验证)：
1. **文献名称**: *NDUFAF4 variants linked to mitochondrial complex I deficiency cause leigh syndrome*
**作者**: Thompson K, et al.
**摘要**: 本研究通过全外显子测序发现NDUFAF4基因突变与线粒体复合物I组装缺陷相关。使用NDUFAF4特异性抗体进行Western blot和免疫荧光实验，证实患者成纤维细胞中NDUFAF4蛋白表达显著降低，导致复合物I功能丧失及Leigh综合征表型。
2. **文献名称**: *The role of NDUFAF4 in mitochondrial respiratory chain biogenesis*
**作者**: Sánchez-Caballero L, et al.
**摘要**: 通过CRISPR敲除细胞模型结合NDUFAF4抗体免疫沉淀，揭示NDUFAF4与复合物I亚基NDUFS1/NDUFV2存在相互作用，证明其作为分子伴侣参与复合物I早期组装阶段，抗体检测显示其缺失导致线粒体膜电位下降。
3. **文献名称**: *Antibody-based profiling of mitochondrial complex I assembly factors in Parkinson's disease*
**作者**: Gergely S, et al.
**摘要**: 研究利用NDUFAF4、NDUFAF6等抗体对帕金森病患者脑组织进行免疫组化分析，发现NDUFAF4蛋白水平与复合物I活性呈正相关，提示其表达异常可能参与神经退行性病变的线粒体功能障碍机制。
**提示**：若需真实文献，建议在PubMed中以“NDUFAF4 antibody”或“NDUFAF4 AND (knockout OR expression)”为关键词检索，重点关注功能研究与疾病模型类论文的方法学部分。
The NDUFAF4 antibody is designed to detect the NDUFAF4 protein, a critical assembly factor for mitochondrial Complex I (NADH:ubiquinone oxidoreductase), the largest enzyme in the electron transport chain. NDUFAF4. also known as NADH dehydrogenase [ubiquinone] 1 alpha subcomplex assembly factor 4. plays a role in the early stages of Complex I biogenesis by stabilizing intermediate structures and ensuring proper subunit integration. Mutations in the NDUFAF4 gene are linked to mitochondrial disorders, particularly Leigh syndrome, a severe neurodegenerative condition characterized by progressive motor and respiratory deficits.
Antibodies targeting NDUFAF4 are essential tools in studying Complex I assembly defects, mitochondrial dysfunction, and disease mechanisms. They are widely used in techniques like Western blotting, immunofluorescence, and immunohistochemistry to assess protein expression, localization, and interactions in cellular or tissue samples. Researchers also employ these antibodies to validate gene-editing models (e.g., CRISPR/Cas9 knockouts) or analyze patient-derived samples for diagnostic purposes.
Due to the protein's mitochondrial matrix localization, proper subcellular fractionation or mitochondrial enrichment is often required for optimal detection. Commercial NDUFAF4 antibodies are typically validated for specificity using knockout controls. Understanding NDUFAF4's role and its antibody applications advances research into mitochondrial biology, metabolic disorders, and therapeutic strategies targeting oxidative phosphorylation deficiencies.
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