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  4. Recombinant Human NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial (NDUFS4)

Recombinant Human NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial (NDUFS4)

  • 中文名稱：
    人NDUFS4重組蛋白
  • 貨號：
    CSB-YP015663HU
  • 規格：
  • 來源：
    Yeast
  • 其他：
  • 中文名稱：
    人NDUFS4重組蛋白
  • 貨號：
    CSB-EP015663HU
  • 規格：
  • 來源：
    E.coli
  • 其他：
  • 中文名稱：
    人NDUFS4重組蛋白
  • 貨號：
    CSB-EP015663HU-B
  • 規格：
  • 來源：
    E.coli
  • 共軛：
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他：
  • 中文名稱：
    人NDUFS4重組蛋白
  • 貨號：
    CSB-BP015663HU
  • 規格：
  • 來源：
    Baculovirus
  • 其他：
  • 中文名稱：
    人NDUFS4重組蛋白
  • 貨號：
    CSB-MP015663HU
  • 規格：
  • 來源：
    Mammalian cell
  • 其他：

產品詳情

  • 純度：
    >85% (SDS-PAGE)
  • 基因名：
  • Uniprot No.：
  • 別名：
    AQDQ; CI 18; CI 18 kDa; CI AQDQ; CI-18 kDa; CI-AQDQ; Complex I 18 kDa; Complex I AQDQ; Complex I-18 kDa; Complex I-AQDQ; mitochondrial; mitochondrial respiratory chain complex I (18 KD subunit); NADH coenzyme Q reductase; NADH dehydrogenase (ubiquinone) Fe S protein 4 18kDa; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4; NADH dehydrogenase; NADH ubiquinone oxidoreductase 18 kDa subunit; NADH-ubiquinone oxidoreductase 18 kDa subunit; NDUFS4; NDUS4_HUMAN
  • 種屬：
    Homo sapiens (Human)
  • 蛋白長度：
    Full Length of Mature Protein
  • 表達區域：
    43-175
  • 氨基酸序列
    AQDQTQDT QLITVDEKLD ITTLTGVPEE HIKTRKVRIF VPARNNMQSG VNNTKKWKME FDTRERWENP LMGWASTADP LSNMVLTFST KEDAVSFAEK NGWSYDIEER KVPKPKSKSY GANFSWNKRT RVSTK
  • 蛋白標簽：
    Tag?type?will?be?determined?during?the?manufacturing?process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 產品提供形式：
    Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 復溶：
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 儲存條件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保質期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 貨期：
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事項：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet ：
    Please contact us to get it.

產品評價

靶點詳情

  • 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.
  • 基因功能參考文獻：
    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 authors 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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  • 相關疾病：
    Mitochondrial complex I deficiency (MT-C1D); Leigh syndrome (LS)
  • 亞細胞定位：
    Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
  • 蛋白家族：
    Complex I NDUFS4 subunit family
  • 數據庫鏈接：

    HGNC: 7711

    OMIM: 252010

    KEGG: hsa:4724

    STRING: 9606.ENSP00000296684

    UniGene: Hs.528222



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