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PIGT Antibody, Biotin conjugated

  • 中文名稱：
    PIGT兔多克隆抗體, Biotin偶聯
  • 貨號：
    CSB-PA836170LD01HU
  • 規格：
    ￥880
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) PIGT Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    PIGT
  • 別名：
    PIGT antibody; CGI-06 antibody; PSEC0163 antibody; UNQ716/PRO1379 antibody; GPI transamidase component PIG-T antibody; Phosphatidylinositol-glycan biosynthesis class T protein antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human GPI transamidase component PIG-T protein (402-562AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Biotin
  • 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
  • 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：
    Component of the GPI transamidase complex. Essential for transfer of GPI to proteins, particularly for formation of carbonyl intermediates.
  • 基因功能參考文獻：
    1. Whole-exome sequencing revealed compound heterozygous mutations (c.250G > T, p.Glu84X and c.1096G > T, p.Gly366Trp) in PIGT (NM_015937.5), which were confirmed using Sanger sequencing. Thus inherited GPI anchor deficiency associated with these PIGT mutations was diagnosed PMID: 28728837
    2. PIGT-knockout HEK293 cells showed that p.(E237Q) results in a small reduction in the amount of CD59 anchored to the cell membrane. PMID: 28327575
    3. mutations in PIGT as the cause of a novel autosomal recessive intellectual disability syndrome PMID: 23636107
    4. Germline mutation and a somatic mutation in PIGT is associated with paroxysmal nocturnal hemoglobinuria. PMID: 23733340
    5. ER-localized because of information in its transmembrane span PMID: 15713669

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  • 相關疾病：
    Multiple congenital anomalies-hypotonia-seizures syndrome 3 (MCAHS3); Paroxysmal nocturnal hemoglobinuria 2 (PNH2)
  • 亞細胞定位：
    Endoplasmic reticulum membrane; Single-pass type I membrane protein.
  • 蛋白家族：
    PIGT family
  • 數據庫鏈接：

    HGNC: 14938

    OMIM: 610272

    KEGG: hsa:51604

    STRING: 9606.ENSP00000279036

    UniGene: Hs.437388



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