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EBP Antibody

  • 货号：
    CSB-PA624016EA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western blot
      All lanes: EBP antibody at 2µg/ml
      Lane 1: A549 whole cell lysate
      Lane 2: 293T whole cell lysate
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 27 kDa
      Observed band size: 27 kDa
    • Immunohistochemistry of paraffin-embedded human breast cancer using CSB-PA624016EA01HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human lung cancer using CSB-PA624016EA01HU at dilution of 1:100
    • Immunofluorescent analysis of 293T cells using CSB-PA624016EA01HU at dilution of 1:100 and 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) EBP Polyclonal antibody
  • Uniprot No.：
    Q15125
  • 基因名：
    EBP
  • 别名：
    EBP; 3-beta-hydroxysteroid-Delta(8,Delta(7-isomerase; Cholestenol Delta-isomerase; Delta(8-Delta(7 sterol isomerase; D8-D7 sterol isomerase; Emopamil-binding protein
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human 3-beta-hydroxysteroid-Delta(8),Delta(7)-isomerase protein (2-230AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，EBP Antibody (CSB-PA624016EA01HU)，的标记方式是Non-conjugated。对于EBP 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-PA624016EB01HU EBP Antibody, HRP conjugated ELISA
    FITC CSB-PA624016EC01HU EBP Antibody, FITC conjugated
    Biotin CSB-PA624016ED01HU EBP 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 1:500-1:2000
    IHC 1:20-1:200
    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：
    Catalyzes the conversion of Delta(8)-sterols to their corresponding Delta(7)-isomers.
  • 基因功能参考文献：
    1. This study expands the current phenotypic spectrum of males with hypomorphic EBP mutations and supports to the hypothesis that there exists an X-linked recessive entity independent of CDPX2. PMID: 24700572
    2. Mutation analysis revealed a heterozygous novel missense mutation, c.204G>T (p.W68C), in exon 2. PMID: 24915996
    3. With non-mosaic EBP mutations in males. PMID: 24459067
    4. Report steroidomimetic aminomethyl spiroacetals as novel inhibitors of the enzyme Delta8,7-sterol isomerase in cholesterol biosynthesis. PMID: 24493593
    5. Elastin binding protein and FKBP65 modulate the kinetics of self-assembly of tropoelastin in an in vitro system. PMID: 24106871
    6. Results show a clear relationship between abnormal sterol profile and the EBP gene mutation PMID: 22121851
    7. postzygotic mosaicism on an ichthyosiform skin lesion in the mother of a girl with X-linked dominant chondrodysplasia punctata associated with a novel EBP mutation. PMID: 21931045
    8. Molecular analysis of EBP mutations were made. PMID: 17378690
    9. Emopamil binding protein (EBP)-shRNA sequences were designed and tested for their effectiveness. PMID: 17498944
    10. We found two novel (3G-->T and 419-422delTTCT) and one known mutation in the EBP gene. The strong phenotypic variability in our patients suggests that there is no clear genotype-phenotype correlation. PMID: 17949453
    11. two unrelated Thai girls with chondrodysplasia punctata type 2. Mutation analysis by PCR-sequencing the entire coding region of emopamil binding protein(EBP) successfully revealed two potentially pathogenic, novel mutations, c.616G-->T and c.382delC. PMID: 18573709

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  • 相关疾病：
    Chondrodysplasia punctata 2, X-linked dominant (CDPX2); MEND syndrome (MEND)
  • 亚细胞定位：
    Endoplasmic reticulum membrane; Multi-pass membrane protein. Nucleus envelope. Cytoplasmic vesicle.
  • 蛋白家族：
    EBP family
  • 数据库链接：

    HGNC: 3133

    OMIM: 300205

    KEGG: hsa:10682

    STRING: 9606.ENSP00000417052

    UniGene: Hs.30619