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Am J Physiol Lung Cell Mol Physiol. 2008 Aug;295(2):L240-63. doi: 10.1152/ajplung.90203.2008. Epub 2008 May 16.

The porcine lung as a potential model for cystic fibrosis.

Author information

1
Department of Internal Medicine, Roy J. Carver College of Medicine, University of Iowa, Iowa City, Iowa 52242, USA.

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.

PMID:
18487356
PMCID:
PMC2519845
DOI:
10.1152/ajplung.90203.2008
[Indexed for MEDLINE]
Free PMC Article

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