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RRAGC Antibody

  • 货号：
    CSB-PA038367
  • 规格：
    ￥1100
  • 图片：
    • Gel: 8%SDS-PAGE, Lysate: 40 μg, Lane 1-2: K562 cells, NIH/3T3 cells, Primary antibody: CSB-PA038367(RRAGC Antibody) at dilution 1/400, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 5 seconds
  • 其他：

产品详情

  • Uniprot No.：
    Q9HB90
  • 基因名：
  • 别名：
    AU041672 antibody; FLJ13311 antibody; GTPase interacting protein 2 antibody; GTPase-interacting protein 2 antibody; GTR2 antibody; MGC47404 antibody; OTTHUMP00000000548 antibody; Rag C antibody; Rag C protein antibody; RAGC antibody; Ras related GTP binding C antibody; Ras-related GTP-binding protein C antibody; RP23 29H22.4 antibody; RRAGC antibody; RRAGC_HUMAN antibody; TIB929 antibody; YGR163W antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse
  • 免疫原：
    Fusion protein of Human RRAGC
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen affinity purification
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB
  • 推荐稀释比：
    Application Recommended Dilution
    ELISA 1:2000-1:5000
    WB 1:500-1:2000
  • 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：
    Guanine nucleotide-binding protein that plays a crucial role in the cellular response to amino acid availability through regulation of the mTORC1 signaling cascade. Forms heterodimeric Rag complexes with RRAGA or RRAGB and cycles between an inactive GTP-bound and an active GDP-bound form. In its active form participates in the relocalization of mTORC1 to the lysosomes and its subsequent activation by the GTPase RHEB. This is a crucial step in the activation of the TOR signaling cascade by amino acids.
  • 基因功能参考文献：
    1. Multiple RRAGC mutations demonstrated elevated MTOR activation. PMID: 27267853
    2. Full-length RagA(GTP):RagC(GDP) dimer binds to regulator to activate mTORC1. PMID: 29107538
    3. The dynamic response of mTORC1 requires intersubunit communication by the Rag GTPases, providing a rationale for why they exist as a dimer and revealing a distinct mode of control for a GTP-binding protein. PMID: 29056322
    4. These findings implicate mTORC1 dysregulation through a gain-of-function mutation in RagC as a novel molecular basis for syndromic forms of pediatric heart failure, and expand genotype-phenotype correlation in RASopathy-related syndromes PMID: 27234373
    5. activating nature of the RRAGC mutations, their existence in the dominant clone and their stability during disease progression support their potential as an excellent candidate for therapeutic targeting PMID: 26691987

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  • 相关疾病：
    RRAGC mutations have been found in a patient with idiopathic dilated cardiomyopathy with ventricular dilation and systolic dysfunction, bilateral cataracts, and mild facial dysmorphisms.
  • 亚细胞定位：
    Cytoplasm. Nucleus. Lysosome.
  • 蛋白家族：
    GTR/RAG GTP-binding protein family
  • 数据库链接：

    HGNC: 19902

    OMIM: 608267

    KEGG: hsa:64121

    STRING: 9606.ENSP00000362092

    UniGene: Hs.532461