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

  1. Home
  2. 抗體
  3. 多克隆抗體
  4. USB1 Antibody

USB1 Antibody

  • 中文名稱(chēng)：
    USB1兔多克隆抗體
  • 貨號(hào)：
    CSB-PA22889A0Rb
  • 規(guī)格：
    ￥440
  • 圖片：
    • IHC image of CSB-PA22889A0Rb diluted at 1:100 and staining in paraffin-embedded human melanoma performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
    • IHC image of CSB-PA22889A0Rb diluted at 1:100 and staining in paraffin-embedded human brain tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱(chēng)：
    Rabbit anti-Homo sapiens (Human) USB1 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    USB1
  • 別名：
    C16orf57 antibody; Chromosome 16 open reading frame 57 antibody; CP057_HUMAN antibody; FLJ13154 antibody; UPF0406 protein C16orf57 antibody
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human U6 snRNA phosphodiesterase protein (1-90AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    Non-conjugated

    本頁(yè)面中的產(chǎn)品，USB1 Antibody (CSB-PA22889A0Rb)，的標(biāo)記方式是Non-conjugated。對(duì)于USB1 Antibody，我們還提供其他標(biāo)記。見(jiàn)下表：

    可提供標(biāo)記
    標(biāo)記方式 貨號(hào) 產(chǎn)品名稱(chēng) 應(yīng)用
    HRP CSB-PA22889B0Rb USB1 Antibody, HRP conjugated ELISA
    FITC CSB-PA22889C0Rb USB1 Antibody, FITC conjugated
    Biotin CSB-PA22889D0Rb USB1 Antibody, Biotin conjugated ELISA
  • 克隆類(lèi)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, IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    IHC 1:20-1:200
  • 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：
    Phosphodiesterase responsible for the U6 snRNA 3' end processing. Acts as an exoribonuclease (RNase) responsible for trimming the poly(U) tract of the last nucleotides in the pre-U6 snRNA molecule, leading to the formation of mature U6 snRNA 3' end-terminated with a 2',3'-cyclic phosphate.
  • 基因功能參考文獻(xiàn)：
    1. this paper describes USB1 mutations characterized in four Moroccan patients out of three unrelated consanguinous families PMID: 28353165
    2. Data indicate the enzymatic activities and structures of yeast and human U6 RNA processing enzyme Usb1. PMID: 28887445
    3. marked overlap of dyskeratosis congenita with four other genetic syndromes, confounding accurate diagnosis and subsequent management. Patients with clinical features of dyskeratosis congenita need to have genetic analysis of USB1, LIG4 and GRHL2 in addition to the classical dyskeratosis congenita genes and telomere length measurements. PMID: 27612988
    4. USB1 genes from myelodysplastic and myelodysplastic/myeloproliferative neoplasms and AML had 3 unreported variants, 2 in USB1 5'UTR (c.-83G>T and c.-66A>G), 1 in IVS3 (c.450-68dupT) and 1 (<1%) in IVS4 (c.587+21A>G/rs200924980) were detected. PMID: 26306619
    5. the link between Mpn1 and snRNA stability PMID: 26213367
    6. Mpn1 associates with the NineTeen Complex, a multiprotein complex that is essential for the maintenance of spliceosome integrity and efficient splicing. [Review] PMID: 23684637
    7. Data indicate that USB1 measures the appropriate length of the U6 oligo(U) tail by reading the position of a key adenine nucleotide (A102) and pausing 5 uridine residues downstream. PMID: 23190533
    8. Recombinant hMpn1 is a 3'-to-5' RNA exonuclease that removes uridines from U6 3' ends, generating terminal 2',3' cyclic phosphates in vitro. PMID: 23022480
    9. characterization of 6 Poikiloderma with Neutropenia patients and mutational repertoire of the gene; detected 2 novel C16orf57 mutations, c.232C>T and c.265 2T>G and the reported c.179delC, c.531delA and c.693 1G>T mutations; bioinformatic prediction of the C16orf57 protein structure denotes a very basic enzymatic function consistent with a housekeeping function PMID: 22269211
    10. We report three cases of poikiloderma with neutropenia whose clinical presentations, laboratory investigations, and C16orf57 mutation support the diagnosis. PMID: 21967010
    11. Advanced bioinformatics predicted that C16orf57 encodes a phosphodiesterase whose putative catalytic activity is essential for its function in vivo PMID: 22899009
    12. Mutations of the C16orf57 gene permit the unification of a distinct group of genetic polikilodermal dermatoses that can be diagnosed as congenital dyskeratosis, Rothmund-Thomson syndrome, poikiloderma-neutropenia. PMID: 21497268
    13. Identification of a novel C16orf57 mutation in Athabaskan patients with Poikiloderma with Neutropenia. PMID: 21271650
    14. findings suggest that mutations in C16orf57 unify a distinct set of families which clinically can be categorized as DC, PN or RTS. PMID: 20817924
    15. report on detailed clinical features of three siblings affected with Clericuzio poikiloderma with neutropenia syndrome, all carrying the same homozygous c.504-2A>Cmutation at the acceptor splice site of intron 4 of C16orf57 gene PMID: 20734427
    16. c16orf57 has a role in clericuzio-type poikiloderma with neutropenia PMID: 20004881

    顯示更多

    收起更多

  • 相關(guān)疾?。?/div>
    Poikiloderma with neutropenia (PN)
  • 亞細(xì)胞定位：
    Nucleus.
  • 蛋白家族：
    2H phosphoesterase superfamily, USB1 family
  • 數(shù)據(jù)庫(kù)鏈接：

    HGNC: 25792

    OMIM: 604173

    KEGG: hsa:79650

    STRING: 9606.ENSP00000219281

    UniGene: Hs.408702



主站蜘蛛池模板： 中文字幕无码中文字幕有码a| 亚欧洲乱码视频一二三区| 欧美熟妇xxxxx欧美老妇不卡 | 青青草原综合久久大伊人精品| 国产成人无码18禁午夜福利p| 国产拍拍拍无码视频免费 | 国产成人久久av免费看| 内射爽无广熟女亚洲| 中文字幕无线码| 极品少妇被黑人白浆直流| 亚洲精品午夜一区人人爽| 夜夜高潮夜夜爽夜夜爱爱一区 | 人人妻人人澡人人爽人人精品av| 在阳台上玩弄人妻的乳球| 亚洲中文字幕第一页在线| 国产精品毛多多水多| 99re热这里只有精品视频| 2020精品自拍视频曝光| 国产精品第一区揄拍无码| 精品欧美一区二区三区久久久| 麻豆国产97在线 | 欧美| 久久99青青精品免费观看| 越南女子杂交内射bbwxz| 国产精品久久久久无码av色戒| 夜夜爽夜夜叫夜夜高潮漏水 | 欧美成人a在线网站| 丰满少妇弄高潮了www| 亚洲精品一区二区丝袜图片| 国产成人a无码短视频| 欧美大胆少妇bbw| 88久久精品无码一区二区毛片| 一本到中文无码av在线精品| 7777色鬼xxxx欧美色妇| 射死你天天日| 一区二区三区高清日本vr| 亚洲色大成网站久久久| 亚洲精品一区三区三区在线观看| 亚洲国产精品18久久久久久| 亚洲国产日韩一区三区| 亚洲国产精品一区二区美利坚| 久久天天躁夜夜躁狠狠躁2022|