Your Good Partner in Biology Research

  1. Home
  2. 抗体
  3. 重组抗体
  4. NDUFS3 Recombinant Monoclonal Antibody

NDUFS3 Recombinant Monoclonal Antibody

  • 货号：
    CSB-RA224121A0HU
  • 规格：
    ￥1320
  • 图片：
    • Western Blot
      Positive WB detected in: 293 whole cell lysate, HepG2 whole cell lysate,
      All lanes: NDUFS3 antibody at 1:2000
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 31, 15 KDa
      Observed band size: 31 kDa
    • IHC image of CSB-RA224121A0HU 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℃ overnight. The primary is detected by a Goat anti-rabbit IgG polymer labeled by HRP and visualized using 0.05% DAB.
  • 其他：

产品详情

  • 产品描述：

    NDUFS3 is a subunit of the NADH: ubiquinone oxidoreductase ETC complex I, which catalyzes the initial stage of electron transfer from NADH to a noncovalently bound flavin mononucleotide and subsequently to the terminal acceptor ubiquinone via a succession of iron-sulfur clusters. NDUFS3 is recruited to the inner mitochondrial membrane to form an early assembly intermediate with NDUFS2 and plays a critical role in the correct assembly of complex I. It starts the complex I assembly process in the mitochondrial matrix. Complex I function is disrupted by NDUFS3 cleavage, resulting in superoxide-dependent but MOMP-independent cell death. NDUFS3 gene missense mutations cause severe encephalomyopathy, including Leigh syndrome (LS).

    CUSABIO designed the vector clones for the expression of a recombinant NDUFS3 antibody in mammalian cells. The vector clones were obtained by inserting the NDUFS3 antibody heavy and light chains into the plasma vectors. The recombinant NDUFS3 antibody was purified from the culture medium through Affinity-chromatography. It can be used to detect NDUFS3 protein from Human in the ELISA, WB, IHC.

  • Uniprot No.：
    O75489
  • 基因名：
  • 别名：
    NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial (EC 1.6.5.3) (EC 1.6.99.3) (Complex I-30kD) (CI-30kD) (NADH-ubiquinone oxidoreductase 30 kDa subunit), NDUFS3
  • 反应种属：
    Human
  • 免疫原：
    A synthesized peptide derived from human NDUFS3
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 克隆类型：
    Monoclonal
  • 抗体亚型：
    Rabbit IgG
  • 纯化方式：
    Affinity-chromatography
  • 克隆号：
    1H12
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Rabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
  • 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 1:50-1:200
  • 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. identified a novel Leigh syndrome causing NDUFS3 mutation and expanded the clinical spectrum caused by NDUFS3 mutations in this study. PMID: 30140060
    2. The results suggest that the impaired mitochondrial activity could be due to the broken interaction between DJ-1 and NDUFS3 and that downregulation of DJ-1 in sperm and testes contributes to AS pathogenesis. PMID: 29849492
    3. The NDUFS3 expressed in the temporal cortex in patient with late-onset Alzheimer's disease. PMID: 28242297
    4. High NDUFS3 expression is associated with IgA nephropathy. PMID: 25770168
    5. pH stability of w-t and Leigh syndrome mutant varied at extreme acidic pH. the molten globule like structure of w-t at pH1 was absent in case of the mutant protein. Both the w-t and mutant proteins showed multi-step thermal and Gdn-HCl induced unfolding PMID: 24028823
    6. NDUFS3 is down-regulated in serous ovarian adenocarcinoma PMID: 23446378
    7. Expressions of GRIM-19, NDUFS3, and extracellular matrix elements are correlated with invasive capabilities of breast cancer cell lines. PMID: 23630608
    8. a novel, biomarker potential of a mitochondrial complex I subunit protein, NDUFS3 - as a robust indicator of breast cancer progression and invasiveness as well as of hypoxia/necrosis in clinical specimens of invasive ductal carcinoma, was uncovered. PMID: 21867691
    9. Decreased protein levels of complex I 30-kDa subunit in fetal Down syndrome brains. PMID: 11771736

    显示更多

    收起更多

  • 亚细胞定位：
    Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
  • 蛋白家族：
    Complex I 30 kDa subunit family
  • 数据库链接：

    HGNC: 7710

    OMIM: 603846

    KEGG: hsa:4722

    STRING: 9606.ENSP00000263774

    UniGene: Hs.502528