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  4. TRAPPC11 Antibody, FITC conjugated

TRAPPC11 Antibody, FITC conjugated

  • 中文名稱：
    TRAPPC11兔多克隆抗體, FITC偶聯
  • 貨號：
    CSB-PA801799LC01HU
  • 規格：
    ￥880
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) TRAPPC11 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    TRAPPC11
  • 別名：
    TRAPPC11 antibody; C4orf41 antibody; Trafficking protein particle complex subunit 11 antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human Trafficking protein particle complex subunit 11 protein (704-836AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    FITC
  • 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
  • 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 endoplasmic reticulum to Golgi apparatus trafficking at a very early stage.
  • 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. The identified novel TRAPPC11 mutation represents an expansion of the myopathy phenotype described before and is characterised particularly by achalasia, alacrima, neurological and muscular phenotypes. PMID: 27707803
    3. TRAPPC11 role in protein glycosylation and lipid-linked oligosaccharides biosynthesis PMID: 26912795
    4. Homozygous mutations in the membrane trafficking component TRAPPC11 causes a form of autosomal-recessive, slowly progressive limb girdle muscular dystrophy with childhood onset and high serum creatine kinase. PMID: 23830518
  • 相關疾病：
    Limb-girdle muscular dystrophy 2S (LGMD2S)
  • 亞細胞定位：
    Golgi apparatus. Golgi apparatus, cis-Golgi network.
  • 蛋白家族：
    TRAPPC11 family
  • 數據庫鏈接：

    HGNC: 25751

    OMIM: 614138

    KEGG: hsa:60684

    STRING: 9606.ENSP00000335371

    UniGene: Hs.443240



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