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PIGV Antibody, FITC conjugated

  • 中文名稱：
    PIGV兔多克隆抗體, FITC偶聯
  • 貨號：
    CSB-PA868321LC01HU
  • 規格：
    ￥880
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) PIGV Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    PIGV
  • 別名：
    GPI mannosyltransferase 2 antibody; GPI mannosyltransferase II antibody; GPI MT II antibody; GPI-MT-II antibody; Phosphatidylinositol glycan biosynthesis class V protein antibody; Phosphatidylinositol-glycan biosynthesis class V protein antibody; PIG-V antibody; Pigv antibody; PIGV_HUMAN antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human GPI mannosyltransferase 2 protein (400-469AA)
  • 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：
    Alpha-1,6-mannosyltransferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers the second mannose to the glycosylphosphatidylinositol during GPI precursor assembly.
  • 基因功能參考文獻：
    1. Data indicate that mannosyltransferases PIGV mutations are the major cause of hyperphosphatasia-mental retardation syndrome (HPMRS) which displays a broad clinical variability regarding associated malformations and growth patterns. PMID: 24129430
    2. PIGV is the rate-limiting enzyme in GPI biosynthesis under limited dolicholphosphate mannose availability. PMID: 23694781
    3. Hyperphosphatasia resulted from secretion of ALP, a GPI-anchored protein normally expressed on the cell surface, into serum due to PIGV deficiency. PMID: 22228761
    4. novel compound heterozygous mutations in the PIGV gene c.467G>A and c.1022C>A and a homozygous mutation c.1022C>A in hyperphosphatasia-mental retardation syndrome PMID: 21739589
    5. PIGV mutations are associated with hyperphosphatasia mental retardation syndrome. PMID: 20802478
    6. PIG-V is the second mannosyltransferase in GPI anchor biosynthesis. PMID: 15623507

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  • 相關疾?。?/div>
    Hyperphosphatasia with mental retardation syndrome 1 (HPMRS1)
  • 亞細胞定位：
    Endoplasmic reticulum membrane; Multi-pass membrane protein.
  • 蛋白家族：
    PIGV family
  • 數據庫鏈接：

    HGNC: 26031

    OMIM: 239300

    KEGG: hsa:55650

    STRING: 9606.ENSP00000078527

    UniGene: Hs.259605



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