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  4. ESPN Antibody

ESPN Antibody

  • 中文名稱：
    ESPN兔多克隆抗體
  • 貨號：
    CSB-PA007827GA01HU
  • 規格：
    ￥3,900
  • 其他：

產品詳情

  • Uniprot No.：
  • 基因名：
    ESPN
  • 別名：
    ESPN antibody; DFNB36 antibody; LP2654Espin antibody; Autosomal recessive deafness type 36 protein antibody; Ectoplasmic specialization protein antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human,Mouse,Rat
  • 免疫原：
    Human ESPN
  • 免疫原種屬：
    Homo sapiens (Human)
  • 抗體亞型：
    IgG
  • 純化方式：
    Antigen Affinity purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA,WB
  • Protocols：
  • 儲存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    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.

產品評價

靶點詳情

  • 功能：
    Multifunctional actin-bundling protein. Plays a major role in regulating the organization, dimension, dynamics and signaling capacities of the actin filament-rich microvilli in the mechanosensory and chemosensory cells. Required for the assembly and stabilization of the stereociliary parallel actin bundles. Plays a crucial role in the formation and maintenance of inner ear hair cell stereocilia. Involved in the elongation of actin in stereocilia. In extrastriolar hair cells, required for targeting MYO3B to stereocilia tips, and for regulation of stereocilia diameter and staircase formation.
  • 基因功能參考文獻：
    1. A significant inverse correlation was observed between miR-612 and Espin protein expression in melanoma tissues. PMID: 29385671
    2. The utricles were then treated with a gamma-secretase inhibitor, followed by espin or control transduction and histochemistry. Although gamma-secretase inhibition increased the number of HCs, few had stereociliary arrays. In contrast, 46 h after espin1 transduction, a significant increase in hair-bundle-like structures was observed PMID: 26886463
    3. The espin actin-filament-binding site has a major effect on the formation and dynamics of actin bundles. PMID: 24424026
    4. human deafness and vestibular dysfunction co-segregated with either of two frameshift mutations in ESPN. PMID: 15286153
    5. The results further strengthen the causative role of the espin gene in non-syndromic hearing loss and add new insights into espin structure and function. PMID: 15930085
    6. Here, we report a new activity of the espins, one that depends on their enigmatic WH2 domain: the ability to assemble a large actin bundle when targeted to a specific subcellular location. PMID: 16569662
    7. A recessive ESPN mutation causing congenital hearing loss in a Morocan family was reported. PMID: 18973245
    8. Flies with mutations affecting the diaphanous,forked, and CG12026/TMHS genes displayed significant reductions in the amplitude of sound-evoked potentials compared to wild-type flies PMID: 19102128

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  • 相關疾病：
    Deafness, autosomal recessive, 36, with or without vestibular involvement (DFNB36)
  • 亞細胞定位：
    Cytoplasm, cytoskeleton. Cell projection, stereocilium. Cell projection, microvillus.
  • 數據庫鏈接：

    HGNC: 13281

    OMIM: 606351

    KEGG: hsa:83715

    STRING: 9606.ENSP00000367059

    UniGene: Hs.744222



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