At present, the only animal models for cystic fibrosis are mice. These animals are excellent models for the intestinal disease of CF, the nasal potential difference mimics the human counterpart, and there are great similarities between the spontaneous human lung infection and the created infection models in the mice with disabling mutations in Cftr. However, these mice are difficult to breed and fragile. We have created the Animal Core to assure availability of this important model for our investigators. The goals of the Animal Core are: 1) to provide well-characterized, genetically defined, specific pathogen free mice to investigators for CF-related subjects. 2) to assist investigators either by training or by direct performance in various manipulations, such as creating mouse models of infection (usually with P. aeruginosa), dosing mice with drugs, recording outcome measures such as nasal potential difference, bronchoalveolar lavage, or quantitative lung morphometry 3) to manage records associated with breeding colonies, experimental procedures, or sample storage. These services are provided for both investigators at Case Western Reserve University and other CF investigators around the world. More than 35 investigators outside of Case have received our well-characterized mice during the last five years, as well as 16 investigators at Case. Over the last five years, the value of mouse models of infection in CF has become clear, but so has the steep learning curve required to create them. The Animal Core can either instruct interested investigators in these models or perform the studies for investigators. More than 30 outside investigators either had experiments done by the Core or received training from them, and 13 investigators at Case accessed these services. Many of them took advantage of the assistance with IACUC protocols. We expect to continue this level of activity in the next grant period, both within Case and outside of it. Since CF mice are available commercially only through one source, and there they are cryopreserved and must be brought up for sale, NIH-supported facilities such as ours serve an important need in the community.
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