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PIGT Antibody

  • 货号：
    CSB-PA017983GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    Q969N2
  • 基因名：
    PIGT
  • 别名：
    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
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Human PIGT
  • 免疫原种属：
    Homo sapiens (Human)
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB,IHC
  • 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：
    Component of the GPI transamidase complex. Essential for transfer of GPI to proteins, particularly for formation of carbonyl intermediates.
  • 基因功能参考文献：
    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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  • 相关疾病：
    Multiple congenital anomalies-hypotonia-seizures syndrome 3 (MCAHS3); Paroxysmal nocturnal hemoglobinuria 2 (PNH2)
  • 亚细胞定位：
    Endoplasmic reticulum membrane; Single-pass type I membrane protein.
  • 蛋白家族：
    PIGT family
  • 数据库链接：

    HGNC: 14938

    OMIM: 610272

    KEGG: hsa:51604

    STRING: 9606.ENSP00000279036

    UniGene: Hs.437388