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NDUFS2 Antibody

  • 货号：
    CSB-PA015661LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western Blot
      Positive WB detected in: Rat kidney tissue, Mouse heart tissue, Mouse brain tissue, Mouse kidney tissue
      All lanes: NDUFS2 antibody at 6.9μg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 53, 52 kDa
      Observed band size: 53 kDa
    • IHC image of CSB-PA015661LA01HU diluted at 1:200 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • IHC image of CSB-PA015661LA01HU diluted at 1:200 and staining in paraffin-embedded human brain tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) NDUFS2 Polyclonal antibody
  • Uniprot No.：
    O75306
  • 基因名：
  • 别名：
    CI 49 antibody; CI 49kD antibody; CI-49kD antibody; Complex 1, mitochondrial respiratory chain, 49 KD subunit antibody; Complex I 49kD antibody; Complex I 49kDa subunit antibody; Complex I-49kD antibody; mitochondrial antibody; NADH dehydrogenase (ubiquinone) Fe S protein 2 49kDa antibody; NADH dehydrogenase (ubiquinone) Fe S protein 2, 49kDa (NADH coenzyme Q reductase) antibody; NADH dehydrogenase [ubiquinone] iron sulfur protein 2, mitochondrial antibody; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2 antibody; NADH ubiquinone oxidoreductase 49 kDa subunit antibody; NADH ubiquinone oxidoreductase NDUFS2 subunit antibody; NADH-ubiquinone oxidoreductase 49 kDa subunit antibody; NADH:ubiquinone oxidoreductase core subunit S2 antibody; Ndufs2 antibody; NDUS2_HUMAN antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human, Rat, Mouse
  • 免疫原：
    Recombinant Human NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial protein (297-427AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，NDUFS2 Antibody (CSB-PA015661LA01HU)，的标记方式是Non-conjugated。对于NDUFS2 Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA015661LB01HU NDUFS2 Antibody, HRP conjugated ELISA
    FITC CSB-PA015661LC01HU NDUFS2 Antibody, FITC conjugated
    Biotin CSB-PA015661LD01HU NDUFS2 Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, WB, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:20-1:200
  • 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 of complex I. Essential for the assembly of complex I. Redox-sensitive, critical component of the oxygen-sensing pathway in the pulmonary vasculature which plays a key role in acute pulmonary oxygen-sensing and hypoxic pulmonary vasoconstriction. Plays an important role in carotid body sensing of hypoxia. Essential for glia-like neural stem and progenitor cell proliferation, differentiation and subsequent oligodendrocyte or neuronal maturation.
  • 基因功能参考文献：
    1. compound heterozygosity for severe and hypomorphic NDUFS2 mutations can cause non-syndromic hereditary optic neuropathy. PMID: 28031252
    2. NDUFAF7 methylates arginine 85 in the NDUFS2 subunit of human complex I. PMID: 24089531
    3. study describes clinical, radiological, biochemical and molecular data of 6 patients with Leigh syndrome with novel mutations in NDUFV1 and NDUFS2; 2 siblings were compound heterozygotes for an undescribed E104A mutation in NDUFS2 PMID: 23266820
    4. The NDUFS2 mutation affects complex I enzymatic function. PMID: 22036843
    5. Our results confirm that NDUFS2 is a mutational hotspot in Caucasian children with isolated complex I deficiency and recommend the routine diagnostic investigation of this gene in patients with Leigh or Leigh-like phenotypes. PMID: 20819849

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  • 相关疾病：
    Mitochondrial complex I deficiency (MT-C1D)
  • 亚细胞定位：
    Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
  • 蛋白家族：
    Complex I 49 kDa subunit family
  • 数据库链接：

    HGNC: 7708

    OMIM: 252010

    KEGG: hsa:4720

    STRING: 9606.ENSP00000356972

    UniGene: Hs.173611