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, HRP conjugated

  • 中文名稱:
    EXOSC3兔多克隆抗體, HRP偶聯(lián)
  • 貨號(hào):
    CSB-PA889083LB01HU
  • 規(guī)格:
    ¥880
  • 其他:

產(chǎn)品詳情

  • 產(chǎn)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(yīng)種屬:
    Human
  • 免疫原:
    Recombinant Human Exosome complex component RRP40 protein (2-275AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標(biāo)記方式:
    HRP
  • 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(chǎn)品提供形式:
    Liquid
  • 應(yīng)用范圍:
    ELISA
  • Protocols:
  • 儲(chǔ)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(chǎn)品評(píng)價(jià)

靶點(diǎn)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(xiàn):
    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(guān)疾病:
    Pontocerebellar hypoplasia 1B (PCH1B)
  • 亞細(xì)胞定位:
    Cytoplasm. Nucleus, nucleolus. Nucleus.
  • 蛋白家族:
    RRP40 family
  • 數(shù)據(jù)庫(kù)鏈接:

    HGNC: 17944

    OMIM: 606489

    KEGG: hsa:51010

    STRING: 9606.ENSP00000323046

    UniGene: Hs.602571



主站蜘蛛池模板: 狠狠色婷婷久久一区二区三区| 国产欧美日韩精品专区| 日本国产制服丝袜一区| 狠狠97人人婷婷五月| 肉大榛一进一出免费视频| 亚洲制服有码在线丝袜| 亚洲乱码中文字幕综合234| 66亚洲一卡2卡新区成片发布| 又大又黄又粗又爽的免费视频| 无遮挡午夜男女xx00动态| 尤物tv国产精品看片在线| 肥臀熟女一区二区三区| 国产乱国产乱老熟300部视频| 99热成人精品热久久6网站| 午夜爱爱爱爱爽爽爽网站| 亚洲a∨无码男人的天堂| 中文无码一区二区不卡av| 无码国产玉足脚交极品播放| 久久av高潮av无码av| 野外少妇愉情中文字幕| 8av国产精品爽爽ⅴa在线观看| 国产杨幂丝袜av在线播放| 无码人妻丰满熟妇a片护士| 亚洲精品97久久中文字幕无码| 亚洲精品自偷自拍无码| 久久天天躁狠狠躁夜夜2o2o| 东北女人啪啪对白| 欧美国产日产一区二区| 天堂在线最新版www中文| 中文无码人妻有码人妻中文字幕 | 日本日本熟妇中文在线视频| 久久精品国产亚洲7777| 国产又色又爽无遮挡免费| 亚洲综合无码一区二区痴汉| 最近中文av字幕在线中文| 亚洲精品乱码久久久久久不卡| 青青草国产线观看| 999精品无码a片在线1级| 久久亚洲一区二区三区四区 | 日韩国产网曝欧美第一页| 亚洲欧美日韩在线一区|