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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

  • 中文名稱：
    人NADH-泛醌氧化還原酶鏈6(MT-ND6)酶聯(lián)免疫試劑盒
  • 貨號：
    CSB-EL015082HU
  • 規(guī)格：
    96T/48T
  • 價格：
    ￥3600/￥2500
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品描述：
    人NADH-泛醌氧化還原酶鏈6(MT-ND6)酶聯(lián)免疫試劑盒（CSB-EL015082HU）為雙抗夾心法ELISA試劑盒，定量檢測血清、血漿、組織勻漿、細(xì)胞裂解物樣本中的MT-ND6含量。MT-ND6是一個靶點(diǎn)。它在細(xì)胞呼吸鏈中有著重要地位，與氧化磷酸化過程相關(guān)。相關(guān)研究機(jī)制主要聚焦于其在能量代謝中的功能、基因突變對細(xì)胞生理功能的影響，以及與神經(jīng)退行性疾病等病癥的潛在關(guān)聯(lián)，為疾病治療提供新方向。試劑盒檢測范圍為23.44 pg/mL-1500 pg/mL，適用于基礎(chǔ)科研中對線粒體功能的機(jī)制研究，例如探究疾病模型中線粒體能量代謝變化、評估基因編輯或藥物干預(yù)對MT-ND6表達(dá)的影響，也可用于篩選調(diào)節(jié)線粒體呼吸鏈活性的化合物。其廣泛的樣本兼容性支持跨類型實(shí)驗(yàn)設(shè)計(jì)，為細(xì)胞生物學(xué)、病理生理學(xué)及代謝相關(guān)研究提供可靠工具。本品僅用于科研，不用于臨床診斷，產(chǎn)品具體參數(shù)及操作步驟詳見產(chǎn)品說明書。
  • 別名：
    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
  • 縮寫：
  • Uniprot No.：
  • 種屬：
    Homo sapiens (Human)
  • 樣本類型：
    serum, plasma, tissue homogenates, cell lysates
  • 檢測范圍：
    23.44 pg/mL-1500 pg/mL
  • 靈敏度：
    5.86 pg/mL
  • 反應(yīng)時間：
    1-5h
  • 樣本體積：
    50-100ul
  • 檢測波長：
    450 nm
  • 研究領(lǐng)域：
    Signal Transduction
  • 測定原理：
    quantitative
  • 測定方法：
    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.
  • 線性度：
    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
  • 標(biāo)準(zhǔn)曲線：
    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
  • 數(shù)據(jù)處理：
  • 貨期：
    3-5 working days

產(chǎn)品評價

靶點(diǎn)詳情

  • 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.
  • 基因功能參考文獻(xiàn)：
    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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  • 相關(guān)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)
  • 亞細(xì)胞定位：
    Mitochondrion inner membrane; Multi-pass membrane protein.
  • 蛋白家族：
    Complex I subunit 6 family
  • 數(shù)據(jù)庫鏈接：

    HGNC: 7462

    OMIM: 252010

    KEGG: hsa:4541

    STRING: 9606.ENSP00000354665