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  4. NDUFS2 Recombinant Monoclonal Antibody

NDUFS2 Recombinant Monoclonal Antibody

  • 中文名稱：
    NDUFS2 Recombinant Monoclonal Antibody
  • 貨號：
    CSB-RA922204A0HU
  • 規格：
    ￥1320
  • 圖片：
    • Western Blot
      Positive WB detected in: THP-1 whole cell lysate(30μg), SY5Y whole cell lysate(30μg), A549 whole cell lysate(30μg), Hela whole cell lysate(30μg), HEK293 whole cell lysate(30μg), HepG2 whole cell lysate(30μg), COLO205 whole cell lysate(30μg), Mouse brain tissue lysate(30μg), Rat brain tissue lysate(30μg)
      All lanes: NDUFS2 antibody at 1:1000
      Secondary
      Goat polyclonal to rabbit IgG at 1/40000 dilution
      Predicted band size: 53kDa
      Observed band size: 53 kDa
      Exposure time：30s
    • IHC image of CSB-RA922204A0HU diluted at 1:100 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 Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.
    • IHC image of CSB-RA922204A0HU diluted at 1:100 and staining in paraffin-embedded human gastric cancer 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 Goat anti-rabbit polymer IgG labeled by HRP and visualized using 0.05% DAB.
    • Overlay Peak curve showing HepG2 cells stained with CSB-RA922204A0HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100 for?10min. Then 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (1ug/1*106cells) for 45min at 4℃. The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 35min at 4℃.Control antibody (green line) was Rabbit IgG (1ug/1*106cells) used under the same conditions. Acquisition of >10,000 events was performed.
  • 其他：

產品詳情

  • Uniprot No.：
  • 基因名：
  • 別名：
    NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial (EC 1.6.5.3) (EC 1.6.99.3) (Complex I-49kD) (CI-49kD) (NADH-ubiquinone oxidoreductase 49 kDa subunit), NDUFS2
  • 反應種屬：
    Human, Mouse, Rat
  • 免疫原：
    A synthesized peptide derived from Human NDUFS2 protein
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated
  • 克隆類型：
    Monoclonal
  • 抗體亞型：
    Rabbit IgG
  • 純化方式：
    Affinity-chromatography
  • 克隆號：
    3C1
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA, WB, IHC, FC
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:50-1:200
    FC 1:20-1:200
  • Protocols：
  • 儲存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    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.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 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 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.
  • 基因功能參考文獻：
    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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  • 相關疾病：
    Mitochondrial complex I deficiency (MT-C1D)
  • 亞細胞定位：
    Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
  • 蛋白家族：
    Complex I 49 kDa subunit family
  • 數據庫鏈接：

    HGNC: 7708

    OMIM: 252010

    KEGG: hsa:4720

    STRING: 9606.ENSP00000356972

    UniGene: Hs.173611



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