Logo image
The porcine lung as a potential model for cystic fibrosis
Journal article   Open access   Peer reviewed

The porcine lung as a potential model for cystic fibrosis

Christopher S Rogers, William M Abraham, Kim A Brogden, John F Engelhardt, John T Fisher, Paul B McCray, Geoffrey McLennan, David K Meyerholz, Eman Namati, Lynda S Ostedgaard, …
American journal of physiology. Lung cellular and molecular physiology, Vol.295(2), pp.L240-L263
08/2008
DOI: 10.1152/ajplung.90203.2008
PMCID: PMC2519845
PMID: 18487356
url
https://doi.org/10.1152/ajplung.90203.2008View
Published (Version of record) Open Access

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.
Review

Details

Metrics

Logo image