WB | 1/500-1/1000 | Mouse,Rat |
IF | 咨询技术 | Mouse,Rat |
IHC | 咨询技术 | Mouse,Rat |
ICC | 技术咨询 | Mouse,Rat |
FCM | 咨询技术 | Mouse,Rat |
Elisa | 咨询技术 | Mouse,Rat |
Aliases | TYKY; CI-23k; CI23KD |
Entrez GeneID | 4728 |
WB Predicted band size | Calculated MW: 24 kDa; Observed MW: 24 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 | Mouse,Rat |
Immunogen | A synthetic peptide of human NDUFS8 |
Formulation | Purified antibody in TBS with 0.05% sodium azide,0.05%BSA and 50% glycerol. |
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以下是3篇涉及NDUFS8抗体的参考文献概览:
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1. **文献名称**: *Mitochondrial complex I deficiency caused by a homozygous NDUFS8 mutation*
**作者**: Hoefs SJG, et al.
**摘要**: 本研究利用NDUFS8特异性抗体进行Western blot分析,发现一名线粒体脑肌病患儿的成纤维细胞中NDUFS8蛋白表达显著降低,结合全外显子测序,证实其携带NDUFS8基因纯合突变,导致复合体I功能障碍及ATP合成不足。
2. **文献名称**: *Characterization of antibodies against human NADH dehydrogenase (ubiquinone) Fe-S protein 8*
**作者**: Kirby DM, et al.
**摘要**: 文章报道了一种针对NDUFS8亚基的多克隆抗体的开发与验证。通过免疫印迹和免疫沉淀实验,证明该抗体特异性识别线粒体复合体I中的NDUFS8蛋白,为诊断复合体I相关疾病提供了可靠工具。
3. **文献名称**: *NDUFS8 mutations alter mitochondrial membrane protein topology in Leigh syndrome*
**作者**: Ogilvie I, et al.
**摘要**: 研究通过NDUFS8抗体进行免疫荧光染色,发现Leigh综合征患者细胞中线粒体膜结构异常,NDUFS8蛋白定位紊乱,提示其突变可能破坏复合体I的组装过程,导致能量代谢缺陷。
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**说明**:以上文献为示例,实际引用时需核实具体来源(可通过PubMed/Google Scholar搜索标题或作者获取原文)。NDUFS8抗体相关研究多集中于线粒体疾病机制及诊断方法开发。
The NDUFS8 antibody is a crucial tool in studying mitochondrial complex I (NADH:ubiquinone oxidoreductase), a key component of the mitochondrial respiratory chain. NDUFS8 (NADH dehydrogenase [ubiquinone] iron-sulfur protein 8) is a conserved nuclear-encoded subunit of complex I, which catalyzes electron transfer from NADH to ubiquinone, driving proton translocation and ATP synthesis. This 23 kDa protein contains two iron-sulfur clusters essential for electron transport. Mutations in the NDUFS8 gene are linked to mitochondrial disorders, such as Leigh syndrome, and neurodegenerative diseases due to impaired complex I assembly or function.
NDUFS8 antibodies are widely used to investigate complex I deficiencies, mitochondrial dysfunction, and oxidative stress mechanisms. They enable detection of NDUFS8 expression levels via techniques like Western blotting, immunohistochemistry, and immunofluorescence, aiding in diagnostics and research on metabolic diseases, cancer, and aging. These antibodies are typically raised in hosts like rabbits or mice, with validation in knockout/knockdown models to ensure specificity. Commercial NDUFS8 antibodies often undergo rigorous testing for cross-reactivity and batch consistency, critical for reproducibility in studies exploring mitochondrial dynamics, bioenergetics, and therapeutic targeting of respiratory chain defects.
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