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.

Public Health Relevance

The systemic nature of CF requires in vivo models to study the disease. The ability to manipulate the mouse and its short generation time make it a powerful resource in which to test hypotheses and answer questions. It is also a powerful complement to the CF pig and ferret, which have fewer tools available and are less easy to manipulate, but which more closely resemble some human phenotypes than the mouse.

National Institute of Health (NIH)
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Center Core Grants (P30)
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Special Emphasis Panel (ZDK1-GRB-7 (J1))
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Case Western Reserve University
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Bruscia, Emanuela M; Bonfield, Tracey L (2016) Cystic Fibrosis Lung Immunity: The Role of the Macrophage. J Innate Immun 8:550-563
Jiang, Kai; Jiao, Sen; Vitko, Megan et al. (2016) The impact of Cystic Fibrosis Transmembrane Regulator Disruption on cardiac function and stress response. J Cyst Fibros 15:34-42
VanDevanter, Donald R; Morris, Nathan J; Konstan, Michael W (2016) IV-treated pulmonary exacerbations in the prior year: An important independent risk factor for future pulmonary exacerbation in cystic fibrosis. J Cyst Fibros 15:372-9
VanDevanter, D R; Flume, P A; Morris, N et al. (2016) Probability of IV antibiotic retreatment within thirty days is associated with duration and location of IV antibiotic treatment for pulmonary exacerbation in cystic fibrosis. J Cyst Fibros 15:783-790
Than, B L N; Linnekamp, J F; Starr, T K et al. (2016) CFTR is a tumor suppressor gene in murine and human intestinal cancer. Oncogene 35:4179-87
Hsu, Daniel; Taylor, Patricia; Fletcher, Dave et al. (2016) Interleukin-17 Pathophysiology and Therapeutic Intervention in Cystic Fibrosis Lung Infection and Inflammation. Infect Immun 84:2410-21
VanDevanter, Donald R; Pasta, David J; Konstan, Michael W (2015) Treatment and demographic factors affecting time to next pulmonary exacerbation in cystic fibrosis. J Cyst Fibros 14:763-9
Donnola, Shannon B; Dasenbrook, Elliott C; Weaver, David et al. (2015) Preliminary comparison of normalized T1 and non-contrast perfusion MRI assessments of regional lung disease in cystic fibrosis patients. J Cyst Fibros :
Powers, Scott W; Stark, Lori J; Chamberlin, Leigh A et al. (2015) Behavioral and nutritional treatment for preschool-aged children with cystic fibrosis: a randomized clinical trial. JAMA Pediatr 169:e150636
Sagel, Scott D; Thompson, Valeria; Chmiel, James F et al. (2015) Effect of treatment of cystic fibrosis pulmonary exacerbations on systemic inflammation. Ann Am Thorac Soc 12:708-17

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