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News and Views
Nature Medicine 10, 452 - 454 (2004)
doi:10.1038/nm0504-452
Finally, mice with CF lung disease
Raymond A Frizzell1 & Joseph M Pilewski1
- Raymond A. Frizzell and Joseph M. Pilewski are in the Departments of Cell Biology and Physiology and of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15261, USA. e-mail: frizzell@pitt.edu or e-mail: pilewskijm@msx.upmc.edu
Abstract
Increasing sodium absorption by overexpressing the epithelial sodium channel in mouse airways results in mucus accumulation and inflammation, changes that occur in the lungs of individuals with cystic fibrosis. The development of lung disease in these mice should provide insights into a disease that has long been lacking an animal model (pages 487–493).
Despite advances over the last few decades, patients with cystic fibrosis typically die of obstructive lung disease by their mid-30s. Following discovery of the responsible gene in 1989, the work of many scientists implicated the cystic fibrosis transmembrane conductance regulator (CFTR) as a cAMP-stimulated anion channel that was missing from the apical (lumen-facing) membranes of cystic fibrosis airway cells1.
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