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, Randall S. Prather, Juan R. Sabater, David Anthony Stoltz, Joseph Zabner, Michael J. Welsh

Research output: Contribution to journalReview article

152 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)L240-L263
JournalAmerican Journal of Physiology - Lung Cellular and Molecular Physiology
Volume295
Issue number2
DOIs
StatePublished - Aug 2008

ASJC Scopus subject areas

  • Physiology
  • Pulmonary and Respiratory Medicine
  • Physiology (medical)
  • Cell Biology

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    Rogers, C. S., Abraham, W. M., Brogden, K. A., Engelhardt, J. F., Fisher, J. T., McCray, P. B., McLennan, G., Meyerholz, D. K., Namati, E., Ostedgaard, L. S., Prather, R. S., Sabater, J. R., Stoltz, D. A., Zabner, J., & Welsh, M. J. (2008). The porcine lung as a potential model for cystic fibrosis. American Journal of Physiology - Lung Cellular and Molecular Physiology, 295(2), L240-L263. https://doi.org/10.1152/ajplung.90203.2008