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RANGRF Antibody

  • 货号：
    CSB-PA019319GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    Q9HD47
  • 基因名：
    RANGRF
  • 别名：
    DKFZp686F02139 antibody; HSPC165 antibody; HSPC236 antibody; MGC110973 antibody; MOG 1 antibody; MOG1 antibody; MOG1 homolog antibody; MOG1_HUMAN antibody; Ran guanine nucleotide release factor antibody; Ran-binding protein MOG1 antibody; RanGNRF antibody; RANGRF antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human RANGRF
  • 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,IHC
  • 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：
    May regulate the intracellular trafficking of RAN. Promotes guanine nucleotide release from RAN and inhibits binding of new GTP by preventing the binding of the RAN guanine nucleotide exchange factor RCC1. Regulates the levels of GTP-bound RAN in the nucleus, and thereby plays a role in the regulation of RAN-dependent mitotic spindle dynamics. Enhances the expression of SCN5A at the cell membrane in cardiomyocytes.
  • 基因功能参考文献：
    1. Suggest that p.E61X_RANGRF is a rare genetic variation with an uncertain role in Brugada Syndrome. PMID: 24142675
    2. Compound mutation of CACNA2D1 and RANGRF genes were found. To the best of our knowledge, this is the first comprehensive description of the concurrence of these two mutations and histiocytoid cardiomyopathy. PMID: 24438356
    3. Results suggest that dominant-negative mutations in MOG1 can impair the trafficking of Na(v)1.5 to the membrane, leading to I(Na) reduction and clinical manifestation of Brugada syndrome. PMID: 21447824
    4. Our screening of Nav1.5 cofactor MOG1 uncovered a novel nonsense variant that appeared to be present at a higher frequency among these patients with atrial fibrillation and Brugada syndrome than control subjects. PMID: 21621375
    5. in cardiomyocytes, MOG1 is mostly localized in the cell membrane and co-localized with Nav1.5, indicating that MOG1 is a critical regulator of sodium channel function in the heart PMID: 18184654

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  • 亚细胞定位：
    Nucleus. Cytoplasm, perinuclear region. Cytoplasm. Cell membrane; Peripheral membrane protein; Cytoplasmic side.
  • 蛋白家族：
    MOG1 family
  • 组织特异性：
    Isoform 1 and isoform 2 are ubiquitously expressed. Detected in heart and brain.
  • 数据库链接：

    HGNC: 17679

    OMIM: 607954

    KEGG: hsa:29098

    STRING: 9606.ENSP00000226105

    UniGene: Hs.408233