This session will focus on the utilization of CF animal models for investigation into CF disease manifestations of diabetes, airway clearance, altered intestinal stem cell proliferation and gastrointestinal cancer risk. Studies of CF ferrets, pigs, mice and ex vivo applications (cell or organoid culture) investigate the specific roles of CFTR in beta cell function, mucociliary clearance of the airways, Wnt signaling of stem cells and intestinal tumor suppression. These topics are discussed within the context of the utility and limitations of these animal models for understanding CF disease and its treatment.
Explain the utility of the CF ferret model in understanding the ways that CFTR affects the biology of pancreatic beta cells and its relevance to the major co-morbidity CF-related diabetes.
Describe the utility and limitations of the CF pig model for recapitulating CF airway disease and the outcomes that might alter current treatment of failed airway clearance.
Discuss the contributions and limitations of the CF mouse model for understanding intestinal hyperproliferation and the increased risk of gastrointestinal cancer in CF patients.