Animal Models Core

小鼠模型对生物医学研究越来越有价值，其重要性与囊性纤维化科学特别相关，这一点可以通过大量出版物证明，这些出版物描述了在这些模型上进行的研究，以了解和治疗人类疾病。动物模型核心机构向CF P30研究者提供了多种小鼠品系，包括先前生成和表征的品系，包括Cftr（Cftr[tm 1Unc]）和剑桥（Cftr[tm 1Cam]）Cftr敲除; Cftr-G551 D（Cftr[Tm 1G 551 D]）; Cftr-F508 del品系（Cftr[Tm 1 Kth]）;表达野生型人CFTR的转基因（Tg）品系， Cftr[-/-]背景（hCFTR Cftr[-/-]）和在Cftr[-/-]背景上表达人CFTR-G542 X的类似Tg小鼠系（hCFTR-G542 X Cftr[-/-]）。 除了我们中心现有的这些已建立的小鼠品系外，我们还协助P30研究人员生成和/或繁殖了几种新型CF小鼠模型，包括Cftr-G542 X敲入系（Cftr[Tm 1G 542 X]）和在Cftr[-/-]背景下表达人CFTR-W1282 X的Tg系（hCFTR-W1282 X Cftr[-/-]）。目前正在核心B中构建许多其他鼠系。 Animal Models Core还通过提供功能性CFTR测定来协助CF研究者，包括活体动物的鼻电位差（PD）测量测定以及新鲜切除组织的肠短路电流测定。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鼠类资源，并对CF动物模型进行高级生理和其他测试。这些资源使研究人员能够在体内研究CF疾病机制，并促进对该疾病的实验疗法的临床前评价。因此，动物模型核心是整个P30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.