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LYRM4 Antibody

  • 货号：
    CSB-PA875729LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • IHC image of CSB-PA875729LA01HU diluted at 1:300 and staining in paraffin-embedded human colon cancer 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 biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • IHC image of CSB-PA875729LA01HU diluted at 1:300 and staining in paraffin-embedded human skeletal muscle 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 biotinylated secondary antibody and visualized using an HRP conjugated SP system.
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) LYRM4 Polyclonal antibody
  • Uniprot No.：
    Q9HD34
  • 基因名：
    LYRM4
  • 别名：
    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
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human LYR motif-containing protein 4 protein (1-91AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，LYRM4 Antibody (CSB-PA875729LA01HU)，的标记方式是Non-conjugated。对于LYRM4 Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA875729LB01HU LYRM4 Antibody, HRP conjugated ELISA
    FITC CSB-PA875729LC01HU LYRM4 Antibody, FITC conjugated
    Biotin CSB-PA875729LD01HU LYRM4 Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:200-1:500
  • 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：
    Required for nuclear and mitochondrial iron-sulfur protein biosynthesis.
  • 基因功能参考文献：
    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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  • 相关疾病：
    Combined oxidative phosphorylation deficiency 19 (COXPD19)
  • 亚细胞定位：
    Mitochondrion. Nucleus.
  • 蛋白家族：
    Complex I LYR family
  • 组织特异性：
    Reduced mRNA levels in Friedreich ataxia patients.
  • 数据库链接：

    HGNC: 21365

    OMIM: 613311

    KEGG: hsa:57128

    STRING: 9606.ENSP00000443900

    UniGene: Hs.387755