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Am J Physiol Lung Cell Mol Physiol 295: L240-L263, 2008. First published May 16, 2008; doi:10.1152/ajplung.90203.2008 Free Article
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REVIEW

The porcine lung as a potential model for cystic fibrosis

Christopher S. Rogers,1 William M. Abraham,2 Kim A. Brogden,3 John F. Engelhardt,4 John T. Fisher,4 Paul B. McCray, Jr.,5 Geoffrey McLennan,1 David K. Meyerholz,6 Eman Namati,1 Lynda S. Ostedgaard,1 Randall S. Prather,7 Juan R. Sabater,2 David Anthony Stoltz,1 Joseph Zabner,1 and Michael J. Welsh1,8

1Department of Internal Medicine, 4Department of Anatomy and Cell Biology, 6Department of Pathology, and 5Department of Pediatrics, Roy J. Carver College of Medicine; 3Department of Periodontics and Dows Institute for Dental Research, College of Dentistry; and 8Howard Hughes Medical Institute, University of Iowa, Iowa City, Iowa; 2Division of Pulmonary and Critical Care Medicine, University of Miami at Mount Sinai Medical Center, Miami Beach, Florida; and 7Division 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)




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C. S. Rogers, D. A. Stoltz, D. K. Meyerholz, L. S. Ostedgaard, T. Rokhlina, P. J. Taft, M. P. Rogan, A. A. Pezzulo, P. H. Karp, O. A. Itani, et al.
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A. S. Verkman
From the farm to the lab: the pig as a new model of cystic fibrosis lung disease
Am J Physiol Lung Cell Mol Physiol, August 1, 2008; 295(2): L238 - L239.
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