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  4. AMMECR1 Antibody

AMMECR1 Antibody

说明书
  • 中文名称：
    AMMECR1兔多克隆抗体
  • 货号：
    CSB-PA22559A0Rb
  • 规格：
    ￥440
  • 图片：
    • IHC image of CSB-PA22559A0Rb diluted at 1:200 and staining in paraffin-embedded human liver cancer 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.
    • IHC image of CSB-PA22559A0Rb diluted at 1:200 and staining in paraffin-embedded human testis 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-PA22559A0Rb 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) AMMECR1 Polyclonal antibody
  • Uniprot No.：
    Q9Y4X0
  • 基因名：
    AMMECR1
  • 别名：
    AMMECR1AMME syndrome candidate gene 1 protein antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human AMME syndrome candidate gene 1 protein (146-248AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，AMMECR1 Antibody (CSB-PA22559A0Rb)，的标记方式是Non-conjugated。对于AMMECR1 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-PA22559B0Rb AMMECR1 Antibody, HRP conjugated ELISA
    FITC CSB-PA22559C0Rb AMMECR1 Antibody, FITC conjugated
    Biotin CSB-PA22559D0Rb AMMECR1 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, IF
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:200-1:500
    IF 1:50-1:200
  • Protocols：
    ELISA Protocol
    Immunohistochemistry (IHC) Protocol
    Immunofluorescence (IF) Protocol
  • 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.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

产品评价

靶点详情

  • 基因功能参考文献：
    1. We conclude that AMMECR1 is a critical gene in the pathogenesis of Alport syndrome with intellectual disability (Mental retardation), Midface hypoplasia and Elliptocytosis (AMME), causing midface hypoplasia and elliptocytosis and contributing to early speech and language delay, infantile hypotonia and hearing loss PMID: 27811305
    2. Study provides further evidence that mutated AMMECR1 gene is responsible for this clinically recognizable X-linked condition, Alport syndrome, mental retardation, midface hypoplasia, and elliptocytosis (AMME complex) with variable expressivity. PMID: 28089922
    3. PH0010 from Pyrococcus horikoshii is highly homologous to human AMMECR 1C-terminal region PMID: 15558565
  • 相关疾病：
    Midface hypoplasia, hearing impairment, elliptocytosis, and nephrocalcinosis (MFHIEN); Alport syndrome with mental retardation, midface hypoplasia and elliptocytosis (ATS-MR)
  • 亚细胞定位：
    Nucleus.
  • 数据库链接：

    HGNC: 467

    OMIM: 300194

    KEGG: hsa:9949

    STRING: 9606.ENSP00000262844

    UniGene: Hs.656243

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