The porcine lung as a potential model for cystic fibrosis

1 Department of Internal Medicine, 4 Department of Anatomy and Cell Biology, 6 Department of Pathology, and 5 Department of Pediatrics, Roy J. Carver College of Medicine; 3 Department of Periodontics and Dows Institute for Dental Research, College of Dentistry; and 8 Howard Hughes Medical Institute, University of Iowa, Iowa City, Iowa; 2 Division of Pulmonary and Critical Care Medicine, University of Miami at Mount Sinai Medical Center, Miami Beach, Florida; and 7 Division of Animal Science, University of Missouri-Columbia, Columbia, Missouri ABSTRACT Airway disease currently causes most of the morbidity and mortality in patients with cystic fibrosis (CF). However, understanding the pathogenesis of CF lung disease and developing novel therapeutic strategies have been hampered by the limitations of current models. Although the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) has been targeted in mice, CF mice fail to develop lung or pancreatic disease like that in humans. In many respects, the anatomy, biochemistry, physiology, size, and genetics of pigs resemble those of humans. Thus pigs with a targeted CFTR gene might provide a good model for CF. Here, we review aspects of porcine airways and lung that are relevant to CF. Address for reprint requests and other correspondence: M. J. Welsh, Howard Hughes Medical Institute, 500 EMRB, Roy J. and Lucille A. Carver College of Medicine, Univ. of Iowa, Iowa City, IA 52242 (e-mail: michael-welsh{at}uiowa.edu )