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

  • 中文名稱:
    EXOSC3兔多克隆抗體
  • 貨號:
    CSB-PA007891GA01HU
  • 規格:
    ¥3,900
  • 其他:

產品詳情

  • 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,Mouse,Rat
  • 免疫原:
    Human EXOSC3
  • 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,IHC
  • 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

  • 相關疾病:
    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: 人妻一区二区三区高清av专区| julia无码中文字幕一区| 中国产xxxxa片在线观看| 久久发布国产伦子伦精品| 久久综合九色综合欧美婷婷| 无码人妻一区二区三区免费n鬼逝 好爽好湿好硬好大免费视频 | 女人张开腿让男人桶爽| 国产精品亚洲а∨无码播放| 成 人 社区在线视频| 377p欧洲日本亚洲大胆| 无罩大乳的熟妇正在播放| 精品无码三级在线观看视频| 四虎国产精品亚洲一区久久特色 | 拍真实国产伦偷精品| 精品久久久久久成人av | 真人作爱90分钟免费看视频| 脱岳裙子从后面挺进去在线观看| 玩弄放荡人妇系列av在线网站| 欧美丰满大乳高跟鞋| 自拍偷在线精品自拍偷免费| 国产午夜亚洲精品国产成人小说| 精品无码一区二区三区不卡| 天无日天天射天天视| 久久99久久99久久综合| 最近日本免费观看高清视频| 国产成人精品无码专区| 色综合色狠狠天天综合网| 亚洲精品无码成人av电影网| 亚洲国产果冻传媒av在线观看 | 国产sm调教视频在线观看| 中文字幕人妻被公上司喝醉在线| 久久精品日日躁夜夜躁| 真实的国产乱xxxx| 亚洲日韩激情无码一区| 免费国产成人午夜福利电影| 国产熟妇乱子伦视频在线观看| 亚洲精品国产一区二区精华液| 精品蜜臀av在线天堂| 肉色丝袜足j视频国产| 欧洲精品99毛片免费高清观看| 精品国产免费一区二区三区香蕉 |