Animal Models Core

动物模型核心

基本信息

项目摘要

Mouse models have become increasingly valuable for biomedical research, and their importance is particularly relevant to cystic fibrosis science as attested to by scores of publications describing studies performed on these models as a means to understand and treat the human disease. The Animal Models Core Facility provides a variety of mouse lines to CF P30 investigators, including previously generated and characterized strains that include the UNC (Cftr[tm1Unc]) and Cambridge (Cftr[tm1Cam]) Cftr knockout; Cftr-G551D (Cftr[Tm1G551D]); Cftr-F508del line (Cftr[Tm1Kth]); a transgenic (Tg) line expressing wild type human CFTR on a Cftr[-/-] background (hCFTR Cftr[-/-]), and a similar Tg mouse line expressing a human CFTR-G542X on a Cftr[-/-] background (hCFTR-G542X Cftr[-/-]). In addition to these established mouse lines available at our Center, we have assisted P30 investigators with the generation and/or breeding of several novel CF mouse models, including a Cftr-G542X knock-in line (Cftr[Tm1G542X]) and a Tg line expressing human CFTR-W1282X in a Cftr[-/-] background (hCFTR-W1282X Cftr[-/-]). A number of other murine lines are presently under construction in Core B. The Animal Models Core also assists CF investigators by providing functional CFTR assays, including nasal Potential Difference (PD) measurements assays in live animals as well as intestinal short circuit current assays of freshly excised tissues. The overall purpose of the Animal Models Core is to provide all necessary murine resources for the successful completion of individual P30 projects, and to perform advanced physiologic and other testing of CF animal models. These resources allow investigators to examine CF disease mechanism in vivo, and facilitate preclinical evaluation of experimental therapeutics for the disease. Thus, the Animal Models Core is an indispensable component of the overall P30.
小鼠模型在生物医学研究中变得越来越有价值,它们的重要性与囊性纤维化科学特别相关,许多出版物证明了这一点,这些出版物描述了在这些模型上进行的研究,作为理解和治疗人类疾病的手段。动物模型核心设施为CF P30研究人员提供了各种小鼠系,包括先前生成的和表征的菌株,其中包括UNC (Cftr[tm1Unc])和Cambridge (Cftr[tm1Cam]) Cftr敲除;Cftr-G551D Cftr(检测[Tm1G551D]);Cftr- f508del线(Cftr[Tm1Kth]);一个表达野生型人CFTR的转基因(Tg)系

项目成果

期刊论文数量(0)
专著数量(0)
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会议论文数量(0)
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David M. Bedwell其他文献

Long-term nonsense suppression therapy with NB84 moderates MPS IH disease progression
  • DOI:
    10.1016/j.ymgme.2013.12.106
  • 发表时间:
    2014-02-01
  • 期刊:
  • 影响因子:
  • 作者:
    Gwendolyn G. Gunn;Yanying Dai;Ming Du;Valery Beklakhov;Jeyakumar Kandasamy;Trenton R. Schoeb;Timor Baasov;David M. Bedwell;Kim M. Keeling
  • 通讯作者:
    Kim M. Keeling
The nonsense suppression drug PTC124 restored alpha-<span class="small-caps">l</span>-iduronidase activity and reduces glycosaminoglycan accumulation in MPS IH mice carrying the Idua-W402X mutation
  • DOI:
    10.1016/j.ymgme.2014.12.026
  • 发表时间:
    2015-02-01
  • 期刊:
  • 影响因子:
  • 作者:
    David M. Bedwell;Dan Wang;Ellen M. Welch;Kim M. Keeling
  • 通讯作者:
    Kim M. Keeling

David M. Bedwell的其他文献

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{{ truncateString('David M. Bedwell', 18)}}的其他基金

New Nonsense Suppression Drugs to Treat MPS I
治疗 MPS I 的新型无意义抑制药物
  • 批准号:
    8842247
  • 财政年份:
    2014
  • 资助金额:
    $ 32.52万
  • 项目类别:
Mechanism of Eukaryotic Translation Termination
真核翻译终止机制
  • 批准号:
    7997463
  • 财政年份:
    2009
  • 资助金额:
    $ 32.52万
  • 项目类别:
Suppression of the Idua-W402X mutation in an MPS I-H mouse
MPS I-H 小鼠 Idua-W402X 突变的抑制
  • 批准号:
    7340377
  • 财政年份:
    2007
  • 资助金额:
    $ 32.52万
  • 项目类别:
UAB CFRC Administrative Core
UAB CFRC 行政核心
  • 批准号:
    10673354
  • 财政年份:
    2007
  • 资助金额:
    $ 32.52万
  • 项目类别:
Suppression of the Idua-W402X mutation in an MPS I-H mouse
MPS I-H 小鼠 Idua-W402X 突变的抑制
  • 批准号:
    8015606
  • 财政年份:
    2007
  • 资助金额:
    $ 32.52万
  • 项目类别:
UAB CF Research and Translation Core Center
UAB CF 研究与翻译核心中心
  • 批准号:
    10673353
  • 财政年份:
    2007
  • 资助金额:
    $ 32.52万
  • 项目类别:
Suppression of the Idua-W402X mutation in an MPS I-H mouse
MPS I-H 小鼠 Idua-W402X 突变的抑制
  • 批准号:
    7179609
  • 财政年份:
    2007
  • 资助金额:
    $ 32.52万
  • 项目类别:
Suppression of the Idua-W402X mutation in an MPS I-H mouse
MPS I-H 小鼠 Idua-W402X 突变的抑制
  • 批准号:
    7560341
  • 财政年份:
    2007
  • 资助金额:
    $ 32.52万
  • 项目类别:
UAB CF Research and Translation Core Center
UAB CF 研究与翻译核心中心
  • 批准号:
    10468801
  • 财政年份:
    2007
  • 资助金额:
    $ 32.52万
  • 项目类别:
Mouse Models Core
鼠标模型核心
  • 批准号:
    7288652
  • 财政年份:
    2007
  • 资助金额:
    $ 32.52万
  • 项目类别:

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