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PIGT Antibody

  • 货号:
    CSB-PA836170LA01HU
  • 规格:
    ¥440
  • 促销:
    小规格抗体限时一口价
  • 图片:
    • IHC image of CSB-PA836170LA01HU diluted at 1:500 and staining in paraffin-embedded human brain tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
  • 其他:

产品详情

  • 产品名称:
    Rabbit anti-Homo sapiens (Human) PIGT Polyclonal antibody
  • 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
  • 免疫原:
    Recombinant Human GPI transamidase component PIG-T protein (402-562AA)
  • 免疫原种属:
    Homo sapiens (Human)
  • 标记方式:
    Non-conjugated

    本页面中的产品,PIGT Antibody (CSB-PA836170LA01HU),的标记方式是Non-conjugated。对于PIGT Antibody,我们还提供其他标记。见下表:

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA836170LB01HU PIGT Antibody, HRP conjugated ELISA
    FITC CSB-PA836170LC01HU PIGT Antibody, FITC conjugated
    Biotin CSB-PA836170LD01HU PIGT Antibody, Biotin conjugated ELISA
  • 克隆类型:
    Polyclonal
  • 抗体亚型:
    IgG
  • 纯化方式:
    >95%, Protein G purified
  • 浓度:
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液:
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式:
    Liquid
  • 应用范围:
    ELISA, IHC
  • 推荐稀释比:
    Application Recommended Dilution
    IHC 1:500-1:1000
  • 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