在线日韩日本国产亚洲丨少妇伦子伦情品无吗丨欧美性猛交xxxx免费看蜜桃丨精品人妻系列无码一区二区三区丨亚洲精品无码不卡在线播放

Your Good Partner in Biology Research

GJB4 Antibody, Biotin conjugated

  • 中文名稱:
    GJB4兔多克隆抗體, Biotin偶聯
  • 貨號:
    CSB-PA865136LD01HU
  • 規格:
    ¥880
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) GJB4 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    GJB4
  • 別名:
    GJB4; Gap junction beta-4 protein; Connexin-30.3; Cx30.3
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human Gap junction beta-4 protein (211-266AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    Biotin
  • 克隆類型:
    Polyclonal
  • 抗體亞型:
    IgG
  • 純化方式:
    >95%, Protein G purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA
  • 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:
    Structural component of gap junctions. Gap junctions are dodecameric channels that connect the cytoplasm of adjoining cells. They are formed by the docking of two hexameric hemichannels, one from each cell membrane. Small molecules and ions diffuse from one cell to a neighboring cell via the central pore.
  • 基因功能參考文獻:
    1. GJB4 may be a genetic risk factor for the development of nonsyndromic hearing loss and the data from the present study can be used to direct the clinical evaluation and effectively manage the care of families of children with GJB4. PMID: 25333454
    2. In this study, we found no mutations of GJB4 in two Progressive symmetrical erythrokeratoderma families. PMID: 23678955
    3. Letter: describe erythrokeratodermia variabilis phenotype related to novel mutation in GJB4 gene. PMID: 23037955
    4. Mutation analysis of GJB3 and GJB4 in Chinese patients with erythrokeratodermia variabilis. PMID: 21950330
    5. Bidirectional sequencing of the coding region of GJB4 revealed a novel c.295G>A missense mutation. PMID: 22266302
    6. There were no mutations found in the GJB4 gene and the true pathogenesis of progressive symmetrical erythrokeratodermia remains unknown. PMID: 21198793
    7. A common frameshift mutation and other variants in GJB4 (connexin 30.3): Analysis of hearing impairment families PMID: 11933201
    8. the involvement of connexin gene 30.3 (GJB4) in the etiology of erythrokeratodermia variabilis PMID: 12648223
    9. These results not only provide new insights into epidermal connexin synthesis and polymerization, but also allow a novel molecular explanation for the similarity of EKV phenotypes. PMID: 14583444
    10. Not all clinically diagnosed individuals with erythrokeratoderma variabilis harbor Cx30.3 disease-associated mutations. PMID: 16297190
    11. Five patients with erythrokeratodermia variabilis of Mendes da Costa and progressive symmetric erythrokeratodermia of Gottron had the same mutation in the GJB4 gene causing the amino acid substitution p.Gly12Asp (G12D). PMID: 19291775

    顯示更多

    收起更多

  • 相關疾病:
    Erythrokeratodermia variabilis et progressiva 2 (EKVP2)
  • 亞細胞定位:
    Cell membrane; Multi-pass membrane protein. Cell junction, gap junction.
  • 蛋白家族:
    Connexin family, Beta-type (group I) subfamily
  • 數據庫鏈接:

    HGNC: 4286

    OMIM: 605425

    KEGG: hsa:127534

    STRING: 9606.ENSP00000345868

    UniGene: Hs.351203



主站蜘蛛池模板: 欧美欧洲成本大片免费| 亚洲欧美精品综合在线观看| 天天躁日日躁狠狠躁性色av| 亚欧av无码乱码在线观看性色 | 欧美老妇交乱视频在线观看| 国偷自产一区二区免费视频| 久久久久久国产精品美女| 在线观看国产丝袜控网站 | 欧洲乱码伦视频免费| 国产精品美脚玉足脚交欧美| 国产亚洲综合欧美视频| 国产成_人_综合_亚洲_国产| 荫蒂被男人添的好舒服爽免费视频| 日本老熟妇乱| 国产偷窥盗摄一区二区| 真人床震高潮全部视频免费 | 99久久无色码中文字幕人妻蜜柚| 男女高潮喷水在线观看| 日日摸天天爽天天爽视频| 香蕉久久久久久久av网站| 精品久久国产综合婷婷五月| 国内精品久久人妻互换| 亚洲午夜爱爱香蕉片| 永久免费的无码中文字幕| 天堂网www最新版官网| 一本一道久久a久久精品综合| 久久久久人妻精品一区二区三区| 一本一道av中文字幕无码| 玩弄人妻少妇老师美妇厨房| 任我爽精品视频在线播放| 无码专区天天躁天天躁在线| 97久久超碰精品视觉盛宴| 香蕉久久精品日日躁夜夜躁夏| 亚洲精品白浆高清久久久久久| 久久无码中文字幕无码| 伊人久久久大香线蕉综合直播| 中文天堂国产最新| 欧美老妇bbbwwbbbww| 国产 日韩 中文字幕 制服| 成人天堂资源www在线| 精品国精品自拍自在线|