Author:
Rogers Christopher S.,Abraham William M.,Brogden Kim A.,Engelhardt John F.,Fisher John T.,McCray Paul B.,McLennan Geoffrey,Meyerholz David K.,Namati Eman,Ostedgaard Lynda S.,Prather Randall S.,Sabater Juan R.,Stoltz David Anthony,Zabner Joseph,Welsh Michael J.
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.
Publisher
American Physiological Society
Subject
Cell Biology,Physiology (medical),Pulmonary and Respiratory Medicine,Physiology
Cited by
199 articles.
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