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  4. GOSR2 Antibody

GOSR2 Antibody

  • 中文名稱：
    GOSR2兔多克隆抗體
  • 貨號：
    CSB-PA009678LA01HU
  • 規格：
    ￥440
  • 圖片：
    • Western blot
      All lanes: GOSR2 antibody at 4µg/ml + Jurkat whole cell lysate
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 25, 23 kDa
      Observed band size: 25 kDa
    • Immunohistochemistry of paraffin-embedded human testis tissue using CSB-PA009678LA01HU at dilution of 1:100
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) GOSR2 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    GOSR2
  • 別名：
    2310032N09Rik antibody; 27 kDa Golgi SNARE protein antibody; Bos1 antibody; EPM6 antibody; Golgi SNAP receptor complex member 2 antibody; Golgi SNARE antibody; Gosr2 antibody; GOSR2_HUMAN antibody; Gs27 antibody; Membrin antibody; SNARE antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human Golgi SNAP receptor complex member 2 protein (1-190AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated

    本頁面中的產品，GOSR2 Antibody (CSB-PA009678LA01HU)，的標記方式是Non-conjugated。對于GOSR2 Antibody，我們還提供其他標記。見下表：

    可提供標記
    標記方式 貨號 產品名稱 應用
    HRP CSB-PA009678LB01HU GOSR2 Antibody, HRP conjugated ELISA
    FITC CSB-PA009678LC01HU GOSR2 Antibody, FITC conjugated
    Biotin CSB-PA009678LD01HU GOSR2 Antibody, Biotin conjugated ELISA
  • 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
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA, WB, IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:20-1:200
  • 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.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 功能：
    Involved in transport of proteins from the cis/medial-Golgi to the trans-Golgi network.
  • 基因功能參考文獻：
    1. Recessive mutations in TRAPPC11 and GOSR2 are associated with congenital muscular dystrophy and hypoglycosylation of alpha-dystroglycan. PMID: 29855340
    2. Molecular dynamics (MD) simulations showed that the hydrophobic core, which triggers SNARE complex formation, is compromised due to the glycine-to-tryptophan substitution in both GOSR2 and Bos1. PMID: 28982678
    3. Mutations in GOSR2 reveal stringent secretory pathway demands of dendritic growth and synaptic integrity. PMID: 28978487
    4. review of the phenotype/genotype of GOSR2-associated progressive myoclonus epilepsy [review] PMID: 27618868
    5. The SNAREs(Soluble N-ethylmaleimide-sensitive factor attachment protein receptors), that regulate both the biogenesis and secretion of multiple lysosome-related organelles(LROs). PMID: 26760525
    6. Based on the presented phenotype, we would advise movement disorder specialists to consider mutation analysis of GOSR2 in patients with Ramsay Hunt syndrome, especially when they also have areflexia. PMID: 24458321
    7. A haplotype of the GOSR2 gene is associated with myocardial infarction in Japanese men PMID: 23675987
    8. Single nucleotide polymorphisms in the GOSR2 gene are associated with essential hypertension in Japanese men. PMID: 23313660
    9. GOSR2 gene mutation is associated with progressive myoclonus epilepsy cases, all of whom came from countries bounding the North Sea, extending to the coastal region of Northern Norway. PMID: 23449775
    10. This p.Gly144Trp mutation is equivalent to a loss of function and results in failure of GOSR2 protein to localize to the cis-Golgi. PMID: 21549339
    11. A homozygous mutation in GOSR2 (c.430G>T, p.Gly144Trp), was identified in five apparently unrelated families with a clinically distinct progressive myoclonic epilepsy syndrome. PMID: 21549339
    12. These studies suggest that membrin recruits Arf-1 to the early Golgi and reveal distinct kinetic cycles for Arf-1 at early and late Golgi determined by different sets of Arf regulators and effectors. PMID: 15781476
    13. We found evidence that a SNP in GOSR2 is modestly associated with hypertension in whites from the ARIC study and the WGHS. PMID: 19057520

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  • 相關疾病：
    Epilepsy, progressive myoclonic 6 (EPM6)
  • 亞細胞定位：
    Golgi apparatus, cis-Golgi network membrane; Single-pass type IV membrane protein. Golgi apparatus membrane. Endoplasmic reticulum membrane.
  • 蛋白家族：
    GOSR2 family
  • 數據庫鏈接：

    HGNC: 4431

    OMIM: 604027

    KEGG: hsa:9570

    STRING: 9606.ENSP00000225567

    UniGene: Hs.463278