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NOP56 Antibody

  • 货号：
    CSB-PA015939GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    O00567
  • 基因名：
    NOP56
  • 别名：
    NOP56 antibody; NOL5ANucleolar protein 56 antibody; Nucleolar protein 5A antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human NOP56
  • 免疫原种属：
    Homo sapiens (Human)
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB
  • 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：
    Involved in the early to middle stages of 60S ribosomal subunit biogenesis. Core component of box C/D small nucleolar ribonucleoprotein (snoRNP) particles. Required for the biogenesis of box C/D snoRNAs such U3, U8 and U14 snoRNAs.
  • 基因功能参考文献：
    1. Genetic and clinical analysis of spinocerebellar ataxia type 36 in Mainland China, with its causative mutation as a hexanucleotide GGCCTG repeat expansion in intron 1 of the NOP56 gene, has been described. PMID: 26661328
    2. Studies indicate that the causative mutation for spinocerebellar ataxias SCA36, namely intronic hexanucleotide GGCCTG expansion in NOP56 gene, has been identified. PMID: 26663071
    3. We newly found intronic hexanucleotide GGCCTG gene expansion in NOP56 gene as the causative mutation in nine unrelated Japanese familial hereditary spinocerebellar ataxia patients PMID: 22353375
    4. This study demonistrated that NOP56 mutation is response spinocerebellar ataxia 36 in Spainish family. PMID: 22492559
    5. Expansion of the intronic GGCCTG hexanucleotide repeat in NOP56 causes a unique form of spinocerebellar ataxias, SCA36, which shows not only ataxia but also motor neuron dysfunction. PMID: 21683323
    6. hNop56p functions in the early to middle stages of 60 S subunit synthesis in human cells and has functional similarities with treacle-associated ribonucleoproteins PMID: 12777385
    7. Data demonstrate that fibrillarin and Nop56 directly interact in vivo, and that this interaction is indispensable for the association of both proteins with the box C/D snoRNPs. PMID: 19331828
    8. snoRNP assembly factor NUFIP can regulate the interactions between TIP48 and TIP49 and the core box C/D proteins. PMID: 19620283

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  • 相关疾病：
    Spinocerebellar ataxia 36 (SCA36)
  • 亚细胞定位：
    Nucleus, nucleolus. Cytoplasm. Nucleus, nucleoplasm.
  • 蛋白家族：
    NOP5/NOP56 family
  • 数据库链接：

    HGNC: 15911

    OMIM: 614153

    KEGG: hsa:10528

    STRING: 9606.ENSP00000370589

    UniGene: Hs.376064