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PIGV Antibody

  • 货号:
    CSB-PA868321LA01HU
  • 规格:
    ¥440
  • 促销:
    小规格抗体限时一口价
  • 图片:
    • Western Blot
      Positive WB detected in: A549 whole cell lysate
      All lanes: PIGV antibody at 4.6µg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 56 kDa
      Observed band size: 56 kDa
    • IHC image of CSB-PA868321LA01HU diluted at 1:200 and staining in paraffin-embedded human kidney 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.
    • Immunofluorescence staining of Hela cells with CSB-PA868321LA01HU at 1:66, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他:

产品详情

  • 产品名称:
    Rabbit anti-Homo sapiens (Human) PIGV Polyclonal antibody
  • Uniprot No.:
    Q9NUD9
  • 基因名:
    PIGV
  • 别名:
    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
  • 反应种属:
    Human
  • 免疫原:
    Recombinant Human GPI mannosyltransferase 2 protein (400-469AA)
  • 免疫原种属:
    Homo sapiens (Human)
  • 标记方式:
    Non-conjugated

    本页面中的产品,PIGV Antibody (CSB-PA868321LA01HU),的标记方式是Non-conjugated。对于PIGV 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-PA868321LB01HU PIGV Antibody, HRP conjugated ELISA
    FITC CSB-PA868321LC01HU PIGV Antibody, FITC conjugated
    Biotin CSB-PA868321LD01HU PIGV 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, WB, IHC, IF
  • 推荐稀释比:
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:200-1:500
    IF 1:50-1:200
  • 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:
    Alpha-1,6-mannosyltransferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers the second mannose to the glycosylphosphatidylinositol during GPI precursor assembly.
  • 基因功能参考文献:
    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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  • 相关疾病:
    Hyperphosphatasia with mental retardation syndrome 1 (HPMRS1)
  • 亚细胞定位:
    Endoplasmic reticulum membrane; Multi-pass membrane protein.
  • 蛋白家族:
    PIGV family
  • 数据库链接:

    HGNC: 26031

    OMIM: 239300

    KEGG: hsa:55650

    STRING: 9606.ENSP00000078527

    UniGene: Hs.259605