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MT-ND6 Antibody

  • 货号：
    CSB-PA229750
  • 规格：
    ￥1100
  • 图片：
    • The image on the left is immunohistochemistry of paraffin-embedded Human colon cancer tissue using CSB-PA229750(MT-ND6 Antibody) at dilution 1/40, on the right is treated with synthetic peptide. (Original magnification: ×200)
    • The image on the left is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using CSB-PA229750(MT-ND6 Antibody) at dilution 1/40, on the right is treated with synthetic peptide. (Original magnification: ×200)
  • 其他：

产品详情

  • Uniprot No.：
    P03923
  • 基因名：
  • 别名：
    MT-ND6 antibody; MTND6 antibody; NADH6 antibody; ND6 antibody; NADH-ubiquinone oxidoreductase chain 6 antibody; EC 7.1.1.2 antibody; NADH dehydrogenase subunit 6 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Synthetic peptide of Human MT-ND6
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen affinity purification
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,IHC
  • 推荐稀释比：
    Application Recommended Dilution
    ELISA 1:2000-1:5000
    IHC 1:50-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

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