Mouse Models

鼠标一直是我们程序在活体环境中检查CF问题能力的关键组件。我们已经编制了大量的CF或与CF相关的小鼠模型(超过50个)，并使它们成为同源基因，为研究社区提供同源工具。这一资源已经成为一种国际资源，有近60名调查人员使用核心(16名本地调查人员，38名美国、加拿大、欧洲和澳大利亚的调查人员)。我们已经建立了尽可能不受限制的多边贸易协定，这样就不会阻碍林业研究。我们已经开发了自己的CFR模型，最新的条件(FLOXED)CFTR等位基因，目前正在培育携带人CFTR的小鼠，以允许回答更多的问题。随着CF猪和雪貂的出现，我们使用的小鼠模型也为比较研究提供了机会。与迄今为止所有的CF动物模型一样，CF小鼠是脆弱的，需要特殊的饲养，这是由核心提供的最有效的服务。Cf小鼠模型核心的总体目标是提供Cf的小鼠模型，以了解疾病的病理生理学和开发治疗方法。 为了继续实现我们的目标，CF鼠标机型核心的具体目标是： 向机构、地区、国家和国际CF研究界提供具有良好特色的CF小鼠模型。 加快初步研究的数据收集，并促进涉及CF小鼠模型的实验程序的培训。我们为CF研究人员提供许多服务，包括收集表型数据(如体重、长度、存活时间等)，从CF小鼠品系中获取组织，进行肺部感染和/或药物剂量实验，以及培训CF小鼠操作和护理。这些服务每天由机构用户使用，并经常由外部用户使用。 维护一个中央数据库，其中包含核心中各种CF小鼠品系的繁育历史、观察到的表型和实验记录。

The mouse has been a critical component of our program's ability to examine CF questions in an in vivo setting. We have compiled a large list of CF or CF-related mouse models (over 50) and have made them congenic to provide isogentic tools to the research community. This resource has become an international resource, with nearly 60 investigators using the Core (16 local and 38 across the US, Canada, Europe and Australia). We have set up MTAs that are as non-restrictive as we can make them so that CF research is not impeded. We have developed CF models ourselves, most recently conditional (floxed) alleles of Cftr and are currently generating mice carrying human CFTR to allow additional questions to be answered. With the advent of the CF pig and ferret, the mouse models we use also provide the opportunity for comparative studies. As is apparent with all CF animal models to date, the CF mouse is fragile and requires special husbandry, a service most effectively provided by a core. The overall goal of the CF Mouse Models Core is to provide mouse models of CF for understanding the pathophysiology of the disease and for development of therapies. To continue to accomplish our goals, the specific aims of the CF Mouse Models Core are: To provide well-characterized CF mouse models to the institutional, regional, national and international CF research community. Expedite data collection for preliminary studies and facilitate training in experimental procedures involving CF mouse models. We provide many services to CF investigators that include collecting phenotypic data (e.g., weight, length, survival etc.,) and obtaining tissues from CF mouse strains, carrying out lung infection and/or drug dosing experiments as well as training in CF mouse handling and care. These services are utilized daily by institutional users and frequently by external users. To maintain a centralized database that contains breeding history, phenotypes observed and experimental records of the various CF mouse strains in the Core.