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LYRM4 Antibody, HRP conjugated

  • 中文名稱：
    LYRM4兔多克隆抗體, HRP偶聯(lián)
  • 貨號：
    CSB-PA875729LB01HU
  • 規(guī)格：
    ￥880
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) LYRM4 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    LYRM4
  • 別名：
    BC034664 antibody; C6orf149 antibody; CGI 203 antibody; Chromosome 6 open reading frame 149 antibody; Gm903 antibody; Homolog of yeast Isd11 antibody; ISD11 antibody; LYR motif containing 4 antibody; LYR motif-containing protein 4 antibody; LYRM4 antibody; LYRM4_HUMAN antibody; MGC38622 antibody; Mitochondrial matrix Nfs1 interacting protein antibody
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human LYR motif-containing protein 4 protein (1-91AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    HRP
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    >95%, Protein G purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 產(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA
  • 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.

產(chǎn)品評價

靶點詳情

  • 功能：
    Required for nuclear and mitochondrial iron-sulfur protein biosynthesis.
  • 基因功能參考文獻(xiàn)：
    1. analysis of the NFS1-ISD11-ACP (SDA) complex forms the core of the iron-sulfur (Fe-S) assembly complex and associates with assembly proteins ISCU2, frataxin (FXN), and ferredoxin to synthesize Fe-S clusters PMID: 28634302
    2. Human Isd11 (ISD11) is a helical protein which exists in solution as an equilibrium between monomer, dimeric and tetrameric species when in the absence of human Nfs1 (NFS1). Recombinant ISD11 expressed in E. coli co-purifies with the bacterial orthologue of NFS1, IscS. PMID: 27427956
    3. The NFS1/ISD11 complex further interacts with scaffold protein ISCU and regulator protein frataxin, thereby forming a quaternary complex for Fe-S cluster formation. PMID: 28271877
    4. Molecular dynamics flexible fitting of protein structures docked into the EM map of the model revealed a [FXN(42-210)]24.[NFS1]24.[ISD11]24.[ISCU]24 complex, consistent with the measured 1:1:1:1 stoichiometry of its four components. PMID: 27519411
    5. Our findings highlight that the ISD11 R68A/R68L mutation display reduced affinity to form a stable subcomplex with NFS1, and thereby fails to prevent NFS1 aggregation resulting in impairment of the Fe-S cluster biogenesis PMID: 26342079
    6. Homozygous mutation in LYRM4 was reported in two patients with combined oxidative phosphorylation deficiency. PMID: 23814038
    7. LYRM4 downregulation may be one of the mechanisms involved in inefficient oxidative phosphorylation and oxidative stress, increasingly recognised as contributors to schizophrenia pathogenesis. PMID: 21968932
    8. Strong candidate gene for mitochondrial disease, based on recessive mutations detected in infantile patients PMID: 22277967
    9. Frataxin interacts with ISD11 and multiple mitochondrial chaperones. PMID: 17331979
    10. ISD11 is important in the biogenesis of Fe-S clusters in mammalian cells. PMID: 19454487

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  • 相關(guān)疾?。?/div>
    Combined oxidative phosphorylation deficiency 19 (COXPD19)
  • 亞細(xì)胞定位：
    Mitochondrion. Nucleus.
  • 蛋白家族：
    Complex I LYR family
  • 組織特異性：
    Reduced mRNA levels in Friedreich ataxia patients.
  • 數(shù)據(jù)庫鏈接：

    HGNC: 21365

    OMIM: 613311

    KEGG: hsa:57128

    STRING: 9606.ENSP00000443900

    UniGene: Hs.387755