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

USB1 Antibody, FITC conjugated

  • 中文名稱(chēng):
    USB1兔多克隆抗體, FITC偶聯(lián)
  • 貨號(hào):
    CSB-PA22889C0Rb
  • 規(guī)格:
    ¥880
  • 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:
    FITC
  • 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
  • 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)疾病:
    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: 激情射精爽到偷偷c视频无码| 久久精品国产清自在天天线| 欧美牲交a欧美牲交| аⅴ天堂中文在线网| 小箩莉末发育娇小性色xxxx| 日韩精品一区二区三区vr| 午夜福利麻豆国产精品| 大屁股国产白浆一二区| 久久www免费人成看片入口| av免费网站在线观看| 亚欧洲乱码视频一二三区| 国产人妻人伦精品1国产| 99久久精品国产成人综合 | 亚洲一区二区三区写真| 亚洲人av在线无码影院观看| 天天综合色天天综合色h| 忘忧草影院在线www韩国日本| 三级男人添奶爽爽爽视频| 996热re视频精品视频这里| 亚洲成av人片在线观看无码不卡| 国产极品粉嫩馒头一线天av| 国内精品久久久久影院中文字幕| 在线观看精品视频网站| 国产狂喷潮在线观看| 野外做受又硬又粗又大视频| 成人亚洲精品久久久久| 四虎亚洲精品高清在线观看| 999国内精品永久免费观看| 国产精品久久久久影院老司| 97香蕉碰碰人妻国产欧美| 欧美阿v天堂视频在99线| 国精品人妻无码一区二区三区性色| 在线观看午夜亚洲一区| 欧美精品久久天天躁| 国产精品无码综合区| 4hu四虎永久在线影院| 无套内射在线观看theporn| 无码福利一区二区三区| 一二三区乱码不卡手机版| 国产成人剧情av麻豆果冻| 在线a久青草视频在线观看|