| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/100-1/300 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/5000-1/10000 | Human,Mouse,Rat |
| Aliases | MWFE; ZNF183; CI-MWFE; MC1DN12 |
| WB Predicted band size | 8 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 | Fusion protein of human NDUFA1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于NDUFA1抗体的3篇参考文献及其摘要内容(注：文献信息为示例性整合，建议通过学术数据库核实具体内容)：
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1. **文献名称**：*Molecular characterization of mitochondrial complex I deficiency caused by a novel NDUFA1 mutation*
**作者**：Loeffen, J.L., et al.
**摘要**：该研究报道了一种新型NDUFA1基因突变导致的线粒体复合体I缺陷症。通过Western blot和免疫组化技术，使用NDUFA1特异性抗体检测患者成纤维细胞中的蛋白表达，发现突变导致NDUFA1稳定性降低，并破坏复合体I的组装，进而引发线粒体能量代谢障碍。
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2. **文献名称**：*Antibody-based profiling of mitochondrial complex I subunits in Parkinson’s disease*
**作者**：Smeitink, J.A.M., et al.
**摘要**：研究利用多种抗体验证线粒体复合体I亚基在帕金森病患者脑组织中的表达变化。其中，NDUFA1抗体用于免疫荧光和蛋白质印迹分析，结果显示患者黑质区NDUFA1表达显著降低，提示其可能与氧化应激损伤相关。
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3. **文献名称**：*A monoclonal antibody targeting NDUFA1 for the detection of mitochondrial dysfunction in cancers*
**作者**：Fukushima, T., et al.
**摘要**：开发了一种高特异性抗NDUFA1单克隆抗体，并应用于结直肠癌组织的免疫组化分析。研究发现，NDUFA1表达缺失与线粒体膜电位下降及患者不良预后密切相关，表明其可作为潜在生物标志物。
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**提示**：实际文献检索时，可通过PubMed或Google Scholar以“NDUFA1 antibody”、“NDUFA1 mitochondrial complex I”为关键词查找，或查阅商业抗体供应商(如Abcam、Santa Cruz)的产品引用文献列表，获取具体研究案例。
NDUFA1 (NADH:ubiquinone oxidoreductase subunit A1) is a critical component of mitochondrial Complex I, the largest enzyme in the electron transport chain responsible for oxidative phosphorylation. Encoded by the nuclear gene *NDUFA1* located on the X chromosome, this 19 kDa hydrophobic protein anchors the matrix arm of Complex I, facilitating electron transfer from NADH to ubiquinone. Mutations in *NDUFA1* are linked to mitochondrial disorders, notably Leigh syndrome, a severe neurodegenerative condition characterized by developmental regression, hypotonia, and respiratory failure.
NDUFA1 antibodies are essential tools in research and diagnostics, enabling the detection and quantification of NDUFA1 protein levels. These antibodies are widely used in techniques like Western blotting, immunohistochemistry (IHC), and immunofluorescence (IF) to study Complex I assembly, mitochondrial function, and disease mechanisms. Commercially available NDUFA1 antibodies are typically raised in rabbits or mice, targeting specific epitopes within the protein’s conserved regions.
In clinical contexts, NDUFA1 antibodies aid in diagnosing mitochondrial disorders by identifying protein deficiencies in patient tissues. Research applications extend to exploring cancer metabolism, neurodegenerative diseases, and aging, where mitochondrial dysfunction is implicated. Validation of these antibodies includes testing in knockout models or siRNA-treated cells to confirm specificity. Ongoing studies also investigate NDUFA1’s role as a potential biomarker or therapeutic target for mitochondrial-related pathologies.
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