Animal Models Core

小鼠模型对生物医学研究的价值越来越高，它们的重要性与囊性纤维化科学特别相关，数十篇出版物描述了在这些模型上进行的研究，以此作为了解和治疗人类疾病的手段。动物模型核心设施为CFP30研究人员提供了各种小鼠品系，包括先前产生和鉴定的品系，包括UNC(CFTR[tm1Unc])和Cambridge(CFTR[tm1Cam])CFTR基因敲除；CFTR-G551D(CFTR[Tm1G551D])；CFTR-F508del品系(CFTR[Tm1Kth])；表达野生型人CFTR[Tm1Kth]的转基因(TG)品系 Cftr[-/-]背景(hCFtr cftr[-/-])，以及在cftr[-/-]背景上表达人cftr-G542X的类似的TG小鼠系(hCFtr-G542X cftr[-/-])。 除了我们中心提供的这些已建立的小鼠系外，我们还协助P30研究人员产生和/或培育了几个新的CF小鼠模型，包括CFTR-G542X敲入系(CFTR[Tm1G542X])和在CFTR[-/-]背景下表达人CFTR-W1282X的TG系(hCFTR-W1282X CFTR[-/-])。其他一些小鼠品系目前正在核心B建造中。 动物模型中心还通过提供功能性CFTR分析来协助CF研究人员，包括活体动物的鼻部电位差(PD)测量分析以及新鲜切除组织的肠道短路电流分析。动物模型核心的总体目的是为成功完成单个P30项目提供所有必要的小鼠资源，并对CF动物模型进行高级生理学和其他测试。这些资源使研究人员能够检查活体内的CF疾病机制，并促进对该疾病的实验疗法的临床前评估。因此，动物模型核心是整个P30中不可或缺的组成部分。

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.