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Am J Physiol Lung Cell Mol Physiol (May 16, 2008). doi:10.1152/ajplung.90203.2008 Free Article
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Submitted on February 22, 2008
Revised on April 29, 2008
Accepted on May 14, 2008

The Porcine Lung as a Potential Model for Cystic Fibrosis

Christopher S Rogers1, William M. Abraham2, Kim A Brogden3, John F. Engelhardt1, John T Fisher1, Paul B McCray, Jr.1, Geoffrey McLennan4, David K Meyerholz1, Eman Namati1, Lynda S Ostedgaard1, Randall S. Prather5, Juan R. Sabater, David Anthony Stoltz1, Joseph Zabner1, and Michael J. Welsh6*

1 University of Iowa
2 Mount Sinai Medical Center
3 University of Iowa, Dows Institute for Dental Research
4 The The University of Iowa
5 University of Missouri-ColumbiaUniversity of Missouri-ColumbiaUniversity of Missouri-Columbia
6 HHMI

* To whom correspondence should be addressed. E-mail: michael-welsh{at}uiowa.edu.

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 the porcine lung that are relevant to CF.




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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.
Disruption of the CFTR Gene Produces a Model of Cystic Fibrosis in Newborn Pigs
Science, September 26, 2008; 321(5897): 1837 - 1841.
[Abstract] [Full Text] [PDF]

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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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