Advertisement

Research Article Free access | 10.1172/JCI119861

Lung disease in mice with cystic fibrosis.

G Kent, R Iles, C E Bear, L J Huan, U Griesenbach, C McKerlie, H Frndova, C Ackerley, D Gosselin, D Radzioch, H O'Brodovich, L C Tsui, M Buchwald, and A K Tanswell

Research Institute, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada.

Find articles by Kent, G. in: JCI | PubMed | Google Scholar

Research Institute, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada.

Find articles by Iles, R. in: JCI | PubMed | Google Scholar

Research Institute, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada.

Find articles by Bear, C. in: JCI | PubMed | Google Scholar

Research Institute, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada.

Find articles by Huan, L. in: JCI | PubMed | Google Scholar

Research Institute, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada.

Find articles by Griesenbach, U. in: JCI | PubMed | Google Scholar

Research Institute, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada.

Find articles by McKerlie, C. in: JCI | PubMed | Google Scholar

Research Institute, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada.

Find articles by Frndova, H. in: JCI | PubMed | Google Scholar

Research Institute, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada.

Find articles by Ackerley, C. in: JCI | PubMed | Google Scholar

Research Institute, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada.

Find articles by Gosselin, D. in: JCI | PubMed | Google Scholar

Research Institute, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada.

Find articles by Radzioch, D. in: JCI | PubMed | Google Scholar

Research Institute, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada.

Find articles by O'Brodovich, H. in: JCI | PubMed | Google Scholar

Research Institute, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada.

Find articles by Tsui, L. in: JCI | PubMed | Google Scholar

Research Institute, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada.

Find articles by Buchwald, M. in: JCI | PubMed | Google Scholar

Research Institute, Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada.

Find articles by Tanswell, A. in: JCI | PubMed | Google Scholar

Published December 15, 1997 - More info

Published in Volume 100, Issue 12 on December 15, 1997
J Clin Invest. 1997;100(12):3060–3069. https://doi.org/10.1172/JCI119861.
© 1997 The American Society for Clinical Investigation
Published December 15, 1997 - Version history
View PDF
Abstract

The leading cause of mortality and morbidity in humans with cystic fibrosis is lung disease. Advances in our understanding of the pathogenesis of the lung disease of cystic fibrosis, as well as development of innovative therapeutic interventions, have been compromised by the lack of a natural animal model. The utility of the CFTR-knockout mouse in studying the pathogenesis of cystic fibrosis has been limited because of their failure, despite the presence of severe intestinal disease, to develop lung disease. Herein, we describe the phenotype of an inbred congenic strain of CFTR-knockout mouse that develops spontaneous and progressive lung disease of early onset. The major features of the lung disease include failure of effective mucociliary transport, postbronchiolar over inflation of alveoli and parenchymal interstitial thickening, with evidence of fibrosis and inflammatory cell recruitment. We speculate that the basis for development of lung disease in the congenic CFTR-knockout mice is their observed lack of a non-CFTR chloride channel normally found in CFTR-knockout mice of mixed genetic background.

Version history
  • Version 1 (December 15, 1997): No description
Advertisement
Advertisement