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

EXOSC3 Antibody, Biotin conjugated

  • 中文名稱:
    EXOSC3兔多克隆抗體, Biotin偶聯
  • 貨號:
    CSB-PA889083LD01HU
  • 規格:
    ¥880
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) EXOSC3 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
  • 別名:
    EXOSC3 antibody; RRP40 antibody; CGI-102Exosome complex component RRP40 antibody; Exosome component 3 antibody; Ribosomal RNA-processing protein 40 antibody; p10 antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human Exosome complex component RRP40 protein (2-275AA)
  • 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:
    Non-catalytic component of the RNA exosome complex which has 3'->5' exoribonuclease activity and participates in a multitude of cellular RNA processing and degradation events. In the nucleus, the RNA exosome complex is involved in proper maturation of stable RNA species such as rRNA, snRNA and snoRNA, in the elimination of RNA processing by-products and non-coding 'pervasive' transcripts, such as antisense RNA species and promoter-upstream transcripts (PROMPTs), and of mRNAs with processing defects, thereby limiting or excluding their export to the cytoplasm. The RNA exosome may be involved in Ig class switch recombination (CSR) and/or Ig variable region somatic hypermutation (SHM) by targeting AICDA deamination activity to transcribed dsDNA substrates. In the cytoplasm, the RNA exosome complex is involved in general mRNA turnover and specifically degrades inherently unstable mRNAs containing AU-rich elements (AREs) within their 3' untranslated regions, and in RNA surveillance pathways, preventing translation of aberrant mRNAs. It seems to be involved in degradation of histone mRNA. The catalytic inactive RNA exosome core complex of 9 subunits (Exo-9) is proposed to play a pivotal role in the binding and presentation of RNA for ribonucleolysis, and to serve as a scaffold for the association with catalytic subunits and accessory proteins or complexes. EXOSC3 as peripheral part of the Exo-9 complex stabilizes the hexameric ring of RNase PH-domain subunits through contacts with EXOSC9 and EXOSC5.
  • 基因功能參考文獻:
    1. This is the first case of mitochondrial dysfunction associated with an EXOSC3 mutation, which expands the phenotypic spectrum of pontocerebellar hypoplasia type 1b. PMID: 28687512
    2. Mutations of EXOSC3/Rrp40p associated with pontocerebellar hypoplasia with progressive cerebral atrophy impact ribosomal RNA processing functions of the exosome in S. cerevisiae. PMID: 28053271
    3. EXOSC3 mutations were linked to complicated hereditary spastic paraplegia. PMID: 25149867
    4. study identified new nonsense and missense mutations in the EXOSC3 gene and showed mutations in this gene are exclusively found in pontocerebellar hypoplasia type 1 patients; there are evident genotype-phenotype correlations in EXOSC3-mediated PCH reflected in clinical outcome, age of death and pons hypoplasia PMID: 24524299
    5. The same mutation c.92G-->C, p.G31A in EXOSC3 was found in three unrelated Czech Roma patients with Pontocerebellar hypoplasia type 1 PMID: 23883322
    6. The present study indicates that EXOSC3 mutations can underlie clinical phenotype not classifiable as pontocerebellar hypoplasia type 1. PMID: 23975261
    7. We identified a homozygous mutation [c.395A > C/p.D132A] in EXOSC3 in four patients with muscle hypotonia, developmental delay, spinal anterior horn involvement, and prolonged survival, consistent with the "mild PCH1 phenotype". PMID: 23564332

    顯示更多

    收起更多

  • 相關疾?。?/div>
    Pontocerebellar hypoplasia 1B (PCH1B)
  • 亞細胞定位:
    Cytoplasm. Nucleus, nucleolus. Nucleus.
  • 蛋白家族:
    RRP40 family
  • 數據庫鏈接:

    HGNC: 17944

    OMIM: 606489

    KEGG: hsa:51010

    STRING: 9606.ENSP00000323046

    UniGene: Hs.602571



主站蜘蛛池模板: av网站的免费观看| 蜜臀精品无码av在线播放| 暖暖视频日本| 国产一区二区| 成人综合婷婷国产精品久久| 久久久久99精品成人片牛牛影视| 国产精品久久久久久免费软件| 老司机免费的精品视频| 日本www网站色情乱码| 99久久久无码国产精品不卡 | 国产精品sm捆绑调教视频| 女人爽到高潮视频免费直播 | 最新国产精品久久精品| 午夜成人理论无码电影在线播放 | 国模雨珍浓密毛大尺度150p| 欧美精品一区二区蜜臀亚洲| 任我撸在线视频| 色8久久人人97超碰香蕉987| 亚洲综合精品成人| 欧美性猛交xxxx乱大交蜜桃| 久久综合狠狠综合久久综合88 | 国产a∨精品一区二区三区不卡| 精品久久久噜噜噜久久久| 无码国模国产在线观看免费| 久久99日韩国产精品久久99| 成年美女黄网站18禁免费| 2019nv天堂香蕉在线观看| 国产女人爽的流水毛片| 无码国内精品久久人妻蜜桃| 久天啪天天久久99久孕妇| 97丨九色丨国产人妻熟女| 俄罗斯丰满熟妇hd| 97人妻无码一区| 67194熟妇人妻欧美日韩| 五月婷婷久久草丁香| 99精品久久久久久久婷婷| 亚洲性视频免费视频网站| 日产2021免费一区| 久久久亚洲精品成人| 少妇与黑人一二三区无码| 亚洲国产成人av网站|