Lung disease in mice with cystic fibrosis.

作者：Geraldine M. Kent, Ray K. Iles, Christine E. Bear, Ling‐Jun Huan, Uta Griesenbach, Colin McKerlie, Helena Frndova, Cameron A. Ackerley, Diane R. Gosselin, Danuta Radzioch, Hugh M O'Brodovich, L.‐C. Tsui, Manuel Buchwald, A. Keith Tanswell · 发表于：Journal of Clinical Investigation · 年份：1997 · DOI：10.1172/jci119861 · 被引用次数：168 · 研究领域：Cystic Fibrosis Research Advances、Neonatal Respiratory Health Research、Tracheal and airway disorders

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.