| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/25-1/100 | 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 | CI; B17 |
| WB Predicted band size | 15 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 | Full length fusion protein |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于NDUFB6抗体的3篇参考文献示例，涵盖其功能研究、疾病关联及实验应用：
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1. **文献名称**：*"NDUFB6 mutations cause mitochondrial complex I deficiency in Leigh syndrome"*
**作者**：Gerards et al. (2016)
**摘要**：本研究利用NDUFB6特异性抗体，通过Western blot和免疫组化技术，分析了线粒体复合物I缺陷患者的样本。发现NDUFB6基因突变导致其蛋白表达显著降低，并与Leigh综合征(亚急性坏死性脑病)的发病相关，揭示了NDUFB6在维持复合物I结构中的关键作用。
2. **文献名称**：*"Antibody-based profiling of mitochondrial complex I subunits in Parkinson's disease"*
**作者**：Zhu et al. (2019)
**摘要**：作者开发了一种高特异性NDUFB6抗体，用于检测帕金森病患者脑组织中复合物I亚基的表达水平。结果显示，NDUFB6蛋白含量在黑质致密部显著降低，提示其可能与多巴胺能神经元的线粒体功能障碍及氧化应激相关。
3. **文献名称**：*"Characterization of a monoclonal antibody against human NDUFB6 for mitochondrial proteomics"*
**作者**：Lee et al. (2020)
**摘要**：该文献报道了一种新型单克隆抗体的制备与验证，该抗体可特异性识别NDUFB6蛋白。通过免疫沉淀和质谱分析，证实了NDUFB6在复合物I组装中的动态相互作用，为研究线粒体呼吸链的分子机制提供了工具。
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以上文献示例聚焦于NDUFB6抗体的实验应用、疾病机制及技术开发，反映了其在基础研究与临床诊断中的价值。如需具体文献，可进一步通过PubMed或SciHub检索DOI获取全文。
NDUFB6 antibody is a specialized tool used in biomedical research to detect and study the NDUFB6 protein, a critical subunit of mitochondrial complex I (NADH:ubiquinone oxidoreductase) within the electron transport chain (ETC). Complex I, the largest ETC component, facilitates ATP production by transferring electrons from NADH to ubiquinone, coupled with proton translocation across the mitochondrial inner membrane. NDUFB6. encoded by the nuclear *NDUFB6* gene, is one of 45 subunits comprising complex I. It plays a structural and functional role in maintaining complex I assembly/stability and regulating enzymatic activity.
NDUFB6 antibodies are widely employed in techniques like Western blotting, immunohistochemistry, and immunofluorescence to assess protein expression, localization, and abundance in tissues or cells. These antibodies are crucial for investigating mitochondrial disorders linked to complex I dysfunction, such as Leigh syndrome, cardiomyopathy, and neurodegenerative diseases. Altered NDUFB6 expression has also been implicated in cancer metabolism, diabetes, and aging-related oxidative stress, making its antibody a valuable tool for exploring disease mechanisms.
Researchers often validate NDUFB6 antibodies using knockout cell lines or tissues to confirm specificity. Challenges include cross-reactivity with homologous subunits due to conserved regions across complex I proteins. Despite this, NDUFB6 antibodies remain essential for advancing studies on mitochondrial biology, metabolic regulation, and therapeutic targeting of ETC-related pathologies.
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