Animal Models Core

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

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.