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

GLE1 Antibody, Biotin conjugated

  • 中文名稱:
    GLE1兔多克隆抗體, Biotin偶聯
  • 貨號:
    CSB-PA700651ED01HU
  • 規格:
    ¥880
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) GLE1 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    GLE1
  • 別名:
    GLE 1 antibody; GLE1 antibody; GLE1 like protein antibody; GLE1 like RNA export mediator antibody; GLE1 RNA export mediator homolog antibody; GLE1 RNA export mediator like (yeast) antibody; GLE1-like protein antibody; GLE1_HUMAN antibody; GLE1L antibody; hGLE1 antibody; LCCS 1 antibody; LCCS antibody; LCCS1 antibody; Nucleoporin GLE1 antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human Nucleoporin GLE1 protein (141-380AA)
  • 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:
    Required for the export of mRNAs containing poly(A) tails from the nucleus into the cytoplasm. May be involved in the terminal step of the mRNA transport through the nuclear pore complex (NPC).
  • 基因功能參考文獻:
    1. Pathogenic variants in the GLE1 gene are rare in Chinese ALS patients. PMID: 29398120
    2. Data indicate 2 siblings with a homozygous p.I684T mutation in RNA export mediator (GLE1). PMID: 28657126
    3. These results imply that DBP5, GLE1 and IP6 have a conserved and individual function in the cytoplasmic mRNA expression. Variations in phenotype are due to the difference in each function of DBP5, GLE1 and IPPK in intracellular mRNA metabolism. PMID: 29746542
    4. We identified bi-allelic mutations in GLE1 in two unrelated individuals with motor delays, feeding difficulties, and respiratory insufficiency who survived beyond the perinatal period. Each affected child had missense variants predicted to result in amino acid substitutions near the C-terminus of GLE1 that are predicted to disrupt protein-protein interaction or GLE1 protein targeting. PMID: 28884921
    5. It was concluded that the amyotrophic lateral sclerosis-linked Gle1-c.1965-2A>C mutation generates a protein isoform capable of both Gle1A- and Gle1B-ascribed functions, and thereby uncoupled from normal mechanisms of Gle1 regulation. PMID: 26776475
    6. We also suggest that lethal congenital contracture syndrome 1 (LCCS1) and lethal arthrogryposis with anterior horn disease (LAAHD), the two AMC subtypes related to GLE1, do not have sufficient clinical or molecular differentiation to be considered allelic disorders. Rather, GLE1 mutations cause a variable spectrum of AMC severity including a non-lethal variant described herein PMID: 27684565
    7. Restoration of miR-127-3p and miR-376a-3p counteracts the neoplastic phenotype of giant cell tumor of bone derived stromal cells by targeting COA1, GLE1 and PDIA6. PMID: 26655997
    8. Role for Gle1A during stress granule formation and translation regulation during environmental stress responses is examined. PMID: 25694449
    9. We report the identification of the first heterozygous mutations in GLE1 ever found to be associated with amyotrophic lateral sclerosis. PMID: 25343993
    10. Lethal congenital contracture syndrome 1 and lethal arthrogryposis with anterior horn cell disease are associated with defective Gle1 function during the export of mRNA. [review] PMID: 24275432
    11. Report documents a requirement for Gle1 self-association during mRNA export and uncover molecular defects underlying a lethal human disease lethal congenital contracture syndrome-1. PMID: 24243016
    12. Dbp5, Gle1-IP6 and Nup159: a working model for mRNP export. PMID: 22064466
    13. defective zebrafish GLE1 function in human LCCS1 results in both neurogenic and non-neurogenic defects linked to the apoptosis of proliferative organ precursors PMID: 22357925
    14. The unique carboxyl-terminal 43 amino acid region of the hGle1B isoform mediates binding to the C-terminal non-phenylalanine- glycine region of the nucleoporin hCG1/NPL1. PMID: 16000379
    15. Mutations in mRNA export mediator GLE1 result in fetal motoneuron disease. PMID: 18204449

    顯示更多

    收起更多

  • 相關疾?。?/div>
    Lethal congenital contracture syndrome 1 (LCCS1); Lethal arthrogryposis with anterior horn cell disease (LAAHD)
  • 亞細胞定位:
    Nucleus. Cytoplasm.; [Isoform 1]: Cytoplasm. Nucleus, nuclear pore complex.
  • 蛋白家族:
    GLE1 family
  • 數據庫鏈接:

    HGNC: 4315

    OMIM: 253310

    KEGG: hsa:2733

    STRING: 9606.ENSP00000308622

    UniGene: Hs.522418



主站蜘蛛池模板: 久久免费午夜福利院| 亚洲综合色噜噜狠狠网站超清| 樱花草在线社区www韩国| 中文字幕av在线一二三区| 亚洲午夜免费福利视频| 久久天天躁狠狠躁夜夜av浪潮| 四十如虎的丰满熟妇啪啪| 情侣激情18内射骚话国产| 中文字幕人妻丝袜二区| 久久综合精品国产二区无码| 少妇人妻av毛片在线看| 一二三区乱码不卡手机版| 欧美激情猛片xxxⅹ大3| 国产免费一区二区三区不卡| 日韩一区二区三区无码人妻视频| 国产凹凸在线一区二区| 国产熟女一区二区三区四区五区| 日韩精品a片一区二区三区妖精| 火箭视频在线观看精品| 国产日产欧产精品精品app| 国产成人久久久精品二区三区| 毛片无码免费无码播放| 另类内射国产在线| 在线 无码 中文字幕 强 乱| 亚洲精品无码成人片| 无码人妻一区、二区、三区免费视频| 色欲网天天无码av| 久久欧美一区二区三区性生奴| 国产猛男猛女超爽免费视频| 欧洲vat一区二区三区| 亚洲中文无码mv| 国产成人av男人的天堂| 亚洲国产精品无码一线岛国| 国产又黄又刺激又高潮的网站| 亚洲中文字幕久久精品蜜桃| 无码少妇一区二区浪潮av| 又色又爽又黄18禁美女裸身无遮挡 | 亚洲 丝袜 另类 校园 欧美| 亚洲综合色自拍一区| 国产精品无码a∨果冻传媒| 2020久热爱精品视频在线观看|