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  4. Recombinant Human NFU1 iron-sulfur cluster scaffold homolog, mitochondrial (NFU1)

Recombinant Human NFU1 iron-sulfur cluster scaffold homolog, mitochondrial (NFU1)

  • 中文名稱：
    人NFU1重組蛋白
  • 貨號：
    CSB-YP891972HU
  • 說明書：
  • 規格：
  • 來源：
    Yeast
  • 其他：
  • 中文名稱：
    人NFU1重組蛋白
  • 貨號：
    CSB-EP891972HU
  • 說明書：
  • 規格：
  • 來源：
    E.coli
  • 其他：
  • 中文名稱：
    人NFU1重組蛋白
  • 貨號：
    CSB-EP891972HU-B
  • 說明書：
  • 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NFU1重組蛋白
  • 貨號：
    CSB-BP891972HU
  • 說明書：
  • 規格：
  • 來源：
    Baculovirus
  • 其他：
  • 中文名稱：
    人NFU1重組蛋白
  • 貨號：
    CSB-MP891972HU
  • 說明書：
  • 規格：
  • 來源：
    Mammalian cell
  • 其他：

產品詳情

  • 純度：
    >85% (SDS-PAGE)
  • 基因名：
    NFU1
  • Uniprot No.：
  • 別名：
    0610006G17Rik; CGI 33; HIRA interacting protein 5; HIRA-interacting protein 5; Hirip5; Histone cell cycle regulation defective interacting protein 5; Iron sulfur cluster scaffold protein; MGC142252; MGC142254; mHIRIP5; mitochondrial; Nfu; NFU1; NFU1 iron sulfur cluster scaffold homolog (S. cerevisiae); NFU1 iron sulfur cluster scaffold homolog; NFU1 iron sulfur cluster scaffold homolog, mitochondrial; NFU1 iron-sulfur cluster scaffold homolog; NFU1_HUMAN; NifU; NIFUC; OTTHUMP00000214438; OTTHUMP00000214439; OTTHUMP00000214465; OTTHUMP00000214466; OTTMUSP00000038171; OTTMUSP00000038172
  • 種屬：
    Homo sapiens (Human)
  • 蛋白長度：
    Full Length of Mature Protein
  • 表達區域：
    10-254
  • 氨基酸序列
    G AAAVAAGLRR RFCHMLKNPY TIKKQPLHQF VQRPLFPLPA AFYHPVRYMF IQTQDTPNPN SLKFIPGKPV LETRTMDFPT PAAAFRSPLA RQLFRIEGVK SVFFGPDFIT VTKENEELDW NLLKPDIYAT IMDFFASGLP LVTEETPSGE AGSEEDDEVV AMIKELLDTR IRPTVQEDGG DVIYKGFEDG IVQLKLQGSC TSCPSSIITL KNGIQNMLQF YIPEVEGVEQ VMDDESDEKE ANSP
  • 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：
    Iron-sulfur cluster scaffold protein which can assemble [4Fe-4S] clusters and deliver them to target proteins.
  • 基因功能參考文獻：
    1. data support a hypothesis in which Nfu can serve as an alternative carrier protein for chaperone-mediated cluster release and delivery in Fe-S cluster biogenesis and trafficking PMID: 29211945
    2. NFU1 gene mutations may cause severe mitochondrial respiratory chain defects, mitochondrial encephalomyopathies and death, early in life (Review) PMID: 27381105
    3. Novel NFU1 variants were identified in Chinese patients with multiple mitochondrial dysfunctions syndrome. PMID: 28470589
    4. report the results of an investigation on the effect of these substitutions on both cluster coordination and NFU1 structure and function PMID: 28906593
    5. Gly189Arg substitution on NFU1 is associated with multiple mitochondrial dysfunctions syndrome 1. PMID: 28906594
    6. a direct role for human Nfu in the process of [2Fe-2S] cluster trafficking and delivery PMID: 27538573
    7. Analysis of protein stability and oligomeric state demonstrates that the Gly208Cys mutant increases the propensity to dimerize and perturbs the secondary structure composition. These changes appear to underlie the severely decreased ability of mutant NFU1 to accept an Fe/S cluster from physiologically relevant sources. PMID: 28161430
    8. Study used NMR spectroscopy and small-angle X-ray scattering data to determine the 3D structure of human mitochondrial NFU1 in its apo- and iron-sulfur cluster-containing holo-form. Apo- NFU1 is monomeric, whereas holo-NFU1 consists of a trimer of three [4Fe-4S] cluster-linked dimers. PMID: 27818104
    9. a leaky splicing regulation due to a splice site mutation (c.545+5G>A) that produces small amounts of wild type NFU1 mRNA that might result in enough protein to partially lipoylate and restore the activity of lipoic acid-dependent enzymes and the assembly and activity of complex I. PMID: 26688339
    10. A new NFU1 mutation is reported in a patient presenting with a milder phenotype characterized by a later onset, a slowly progressive spastic paraparesis with relapsing-remitting episodes, mild cognitive impairment and a long survival. PMID: 25758857
    11. This study provides new insights into the molecular bases of NFU1 disease. PMID: 23179554
    12. A fatal mitochondrial disease is associated with defective NFU1 function in the maturation of a subset of mitochondrial Fe-S proteins PMID: 22077971
    13. Mutations in iron-sulfur cluster scaffold genes NFU1 and BOLA3 cause a fatal deficiency of multiple respiratory chain and 2-oxoacid dehydrogenase enzymes [case report] PMID: 21944046
    14. Analyses of genomic DNA, transcripts, and translation products indicate that alternative splicing of a common pre-mRNA results in synthesis of two Nfu isoforms with distinct subcellular localizations. PMID: 12886008
    15. Laforin interacts with HIRIP5. PMID: 12915448
    16. NFU binds to NifS and reduces the persulfide bond on activated NifS (following formation of the persulfide bond by abstraction of S from the Cys amino acid), yielding inorganic sulfide on a time frame that is compatible with Fe-S cluster assembly. PMID: 19146390
    17. NFU is a functionally competent reducing agent for cysteinyl persulfide bond cleavage, releasing inorganic sulfide for incorporation into the iron-sulfur-bound [2Fe-2S] cluster, a reactivity that may be facilitated by flexibility of the C-terminal domain. PMID: 19722697

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  • 相關疾病：
    Multiple mitochondrial dysfunctions syndrome 1 (MMDS1)
  • 亞細胞定位：
    Mitochondrion. Cytoplasm, cytosol.
  • 蛋白家族：
    NifU family
  • 組織特異性：
    Ubiquitous. Expression in adult lung is weak compared to fetal lung.
  • 數據庫鏈接：

    HGNC: 16287

    OMIM: 605711

    KEGG: hsa:27247

    STRING: 9606.ENSP00000387219

    UniGene: Hs.430439



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