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  4. Human NADH-ubiquinone oxidoreductase chain 6(MT-ND6) ELISA kit

Human NADH-ubiquinone oxidoreductase chain 6(MT-ND6) ELISA kit

文献引用 (5) 说明书下载
  • 中文名称：
    人NADH-泛醌氧化还原酶链6(MT-ND6)酶联免疫试剂盒
  • 货号：
    CSB-EL015082HU
  • 规格：
    96T/48T
  • 价格：
    ￥3600/￥2500
  • 其他：
CUSABIO Wins 2024 Bioz Rapid Star™ Award in the ELISA category

产品详情

  • 产品描述：
    本试剂盒（CSB-EL015082HU）采用双抗体夹心法原理，专为定量检测人血清、血浆、组织匀浆及细胞裂解液中的NADH-泛醌氧化还原酶链6（MT-ND6）设计。MT-ND6是线粒体呼吸链复合物I的核心亚基之一，参与细胞能量代谢的氧化磷酸化过程，其表达异常与神经退行性疾病、代谢紊乱及线粒体功能障碍相关。本产品检测范围为23.44-1500 pg/mL，通过预包被特异性捕获抗体与样本中的MT-ND6结合，再经酶标检测抗体形成复合物，最终通过显色反应实现精准定量。该试剂盒适用于基础科研中对线粒体功能的机制研究，例如探究疾病模型中线粒体能量代谢变化、评估基因编辑或药物干预对MT-ND6表达的影响，也可用于筛选调节线粒体呼吸链活性的化合物。其广泛的样本兼容性支持跨类型实验设计，为细胞生物学、病理生理学及代谢相关研究提供可靠工具。
  • 别名：
    MT-ND6 ELISA Kit; MTND6 ELISA Kit; NADH6 ELISA Kit; ND6 ELISA Kit; NADH-ubiquinone oxidoreductase chain 6 ELISA Kit; EC 7.1.1.2 ELISA Kit; NADH dehydrogenase subunit 6 ELISA Kit
  • 缩写：
    MT-ND6
  • Uniprot No.：
  • 种属：
    Homo sapiens (Human)
  • 样本类型：
    serum, plasma, tissue homogenates, cell lysates
  • 检测范围：
    23.44 pg/mL-1500 pg/mL
  • 灵敏度：
    5.86 pg/mL
  • 反应时间：
    1-5h
  • 样本体积：
    50-100ul
  • 检测波长：
    450 nm
  • 研究领域：
    Signal Transduction
  • 测定原理：
    quantitative
  • 测定方法：
    Sandwich
  • 精密度：
    Intra-assay Precision (Precision within an assay): CV%<8%
    Three samples of known concentration were tested twenty times on one plate to assess.
    Inter-assay Precision (Precision between assays): CV%<10%
    Three samples of known concentration were tested in twenty assays to assess.
  • 线性度：
    To assess the linearity of the assay, samples were spiked with high concentrations of Human MT-ND6 in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay.
    SampleSerum(n=4)
    1:1Average %92
    Range %84-98
    1:2Average %86
    Range %80-92
    1:4Average %94
    Range %85-98
    1:8Average %102
    Range %98-106
  • 回收率：
    The recovery of Human MT-ND6 spiked to levels throughout the range of the assay in various matrices was evaluated. Samples were diluted prior to assay as directed in the Sample Preparation section.
    Sample TypeAverage % RecoveryRange
    Serum (n=5) 9488-98
    EDTA plasma (n=4)8983-95
  • 标准曲线：
    These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.
    pg/mlOD1OD2AverageCorrected
    15002.763 2.867 2.815 2.716
    7502.426 2.321 2.374 2.275
    3751.675 1.772 1.724 1.625
    187.51.045 1.115 1.080 0.981
    93.750.527 0.534 0.531 0.432
    46.880.274 0.277 0.276 0.177
    23.440.188 0.178 0.183 0.084
    00.098 0.099 0.099
  • 数据处理：
    ELISA操作视频及标曲绘制软件下载
  • 货期：
    3-5 working days

引用文献

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MT-ND6 antibodies

靶点详情

  • 功能：
    Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) which catalyzes electron transfer from NADH through the respiratory chain, using ubiquinone as an electron acceptor. Essential for the catalytic activity and assembly of complex I.
  • 基因功能参考文献：
    1. Study identify a significant down-expression of MT-ND6 in men with Klinefelter syndrome. PMID: 29333085
    2. The molecular diagnostics by the whole exome sequencing showed a novel de novo (c.1374-2A>C) mutation in the KRT10 gene responsible for the development of IWC (KRT10 defect was confirmed by immunofluorescent study). Concurrently, the m.14484T>C mutation in mitochondrial MTND6 gene (characteristic for Leber's hereditary optic neuropathy or LHON) was detected in patient, his mother and brother PMID: 28944608
    3. This review focuses on the role of mitochondrial genes in causing LHON and therapeutics available for treating the disease. A systematic search has been adopted in various databases using the keywords "LHON," "mitochondria," "ND1," "ND4," "ND6," and "therapy" and the following review on mitochondrial genetics PMID: 29133631
    4. Liver MT-ND6 transcriptional activity and protein expression were decreased in nonalcoholic steatohepatitis, suggesting that the expression of this mitochondrial gene may play an important role in the disease progression. PMID: 22879518
    5. This study shows that m.14484T>C arose independently in a different mtDNA haplogroup and ethnic background in India, which may influence the clinical expression of the disease. PMID: 23674761
    6. These data suggested that the ND6 T14502C variant may modulate the phenotypic manifestation of the G11778A mutation in these Chinese pedigrees. PMID: 20691156
    7. the G14459A mutation is a candidate mutation for maternally inherited dystonia PMID: 20052369
    8. novel mitochondrial DNA nucleotide transversion, C14482A (M64I) was found to cause Leber's hereditary optic neuropathy with visual recovery PMID: 12112086
    9. mitochondrial ND6 may have a role in Leber's hereditary optic neuropathy PMID: 15922297
    10. Free radicals-mediated damage was studied in transmitochondrial cells harboring T14487C mutation in the ND6 gene of mtDNA. PMID: 16337195
    11. Therefore, the coexistence of the A1555G mutation and T14484C mutations in this Chinese family indicate that the A1555G mutation may play a synergistic role in the phenotypic manifestation of LHON associated ND6 T14484C mutation. PMID: 17452034
    12. T14484C and T14502C in ND6 gene are associated with Leber's hereditary optic neuropathy in a Chinese family. PMID: 18440284
    13. Molecular analysis has led to identification of the known T14502C mutation in ND6 gene in Chinese families with Leber's hereditary optic neuropathy. PMID: 19732751
    14. missense mutation 14487T>C resulted in severe encephalopathies ranging from infantile Leigh syndrome to adult-onset progressive myoclonic epilepsy with dystonia in a five-generation family PMID: 20019223

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  • 相关疾病：
    Leber hereditary optic neuropathy (LHON); Leber hereditary optic neuropathy with dystonia (LDYT); Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes syndrome (MELAS); Mitochondrial complex I deficiency (MT-C1D)
  • 亚细胞定位：
    Mitochondrion inner membrane; Multi-pass membrane protein.
  • 蛋白家族：
    Complex I subunit 6 family
  • 数据库链接：

    HGNC: 7462

    OMIM: 252010

    KEGG: hsa:4541

    STRING: 9606.ENSP00000354665

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