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  4. NDUFS4 Recombinant Monoclonal Antibody

NDUFS4 Recombinant Monoclonal Antibody

  • 货号：
    CSB-RA588190A0HU
  • 规格：
    ￥1320
  • 图片：
    • IHC image of CSB-RA588190A0HU diluted at 1:100 and staining in paraffin-embedded human small intestine 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.
    • Overlay Peak curve showing MCF7 cells stained with CSB-RA588190A0HU (red line) at 1:100. The cells were fixed in 4% formaldehyde and permeated by 0.2% TritonX-100. 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：

产品详情

  • Uniprot No.：
    O43181
  • 基因名：
  • 别名：
    NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial (Complex I-18 kDa) (CI-18 kDa) (Complex I-AQDQ) (CI-AQDQ) (NADH-ubiquinone oxidoreductase 18 kDa subunit), NDUFS4
  • 反应种属：
    Human
  • 免疫原：
    A synthesized peptide derived from human NDUFS4
  • 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：
    9H1
  • 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, IHC, FC
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:50-1:200
    FC 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：
    Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.
  • 基因功能参考文献：
    1. The clinical presentations of five individuals of Hutterite descent with Leigh disease are described herein. An identity-by-descent mapping and candidate gene approach was used to identify a novel homozygous c.393dupA frameshift mutation in the NADH dehydrogenase (ubiquinone) Fe-S protein 4 (NDUFS4) gene. PMID: 27671926
    2. The s concluded that NDUFS4-related Leigh syndrome is invariably linked to an early onset severe phenotype that results in early death. PMID: 27079373
    3. The c.462delA deletion led to a complete lack of NDUFS4 peptide in isolated mitochondria, and this deficiency caused an inefficient mitochondrial complex I assembly and Leigh syndrome symptoms. PMID: 24020637
    4. Mutations in the NDUFS4 gene and its subunits are associated with the mitochondrial complex I deficiency. (Review) PMID: 23378164
    5. Elevated expression of CO I and ND4 were associated with gastric tumorigenesis and tumor dedifferentiation PMID: 22407105
    6. Studies indicate that that the functional capacity of complex I depends on phosphorylation and import of subunit NDUFS4 protein. PMID: 21945319
    7. In fibroblast cultures, protein kinase A-mediated phosphorylation of the NDUFS4 subunit of complex I rescues the activity of the oxidatively damaged complex. PMID: 22198267
    8. case Report: A novel mutation in NDUFS4 causes Leigh syndrome in an Ashkenazi Jewish family. PMID: 19107570
    9. REVIEW: Phosphorylation of the NDUFS4 protein, overall respiratory activity, and mutations sassociated with deficiency of complex I. PMID: 11860175
    10. In patients with complex I deficiency, the increased whole-body oxygen consumption rate at rest reflects increased electron transport through the respiratory chain, driven by a decreased phosphorylation potential. PMID: 11940698
    11. observations show the essential role of the 18-kDa subunit of respiratory complex I (NDUFS4) gene in the structure and function of complex I and give insight into pathogenic mechanism of NDUFS4 gene mutations in a severe defect of complex I PMID: 12944388
    12. A nonsense mutation leading to the abrogation of mRNA decay was found in NDUFS4 gene of a Leigh syndrome patient. PMID: 15975579
    13. NDUSF4 is required for the assembly and stabilization of a portion of complex I that contains a number of subunits. PMID: 17438127
    14. impact of PKA mediated phosphorylation on the mitochondrial import of in vitro and in vivo synthesized NDUFS4 protein PMID: 18291624
    15. regulation of alternative transcripts of the NDUFS4 gene of complex I of the respiratory chain PMID: 18555024
    16. NDUFS4 presents a hotspot of mutations in the genetic apparatus of oxidative phosphorylation and the correct assembly of the subunit it encodes is essential for completion of the assembly of complex I. PMID: 19364667

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  • 相关疾病：
    Mitochondrial complex I deficiency (MT-C1D); Leigh syndrome (LS)
  • 亚细胞定位：
    Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
  • 蛋白家族：
    Complex I NDUFS4 subunit family
  • 数据库链接：

    HGNC: 7711

    OMIM: 252010

    KEGG: hsa:4724

    STRING: 9606.ENSP00000296684

    UniGene: Hs.528222