Lung disease in mice with cystic fibrosis
| Autor: | Cameron Ackerley, Geraldine Kent, R. Iles, Danuta Radzioch, Uta Griesenbach, Manuel Buchwald, A. K. Tanswell, Christine E. Bear, Lap-Chee Tsui, Colin McKerlie, Hugh O'Brodovich, L J Huan, Diane Gosselin, Helena Frndova |
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| Jazyk: | angličtina |
| Rok vydání: | 1997 |
| Předmět: |
Male
Pathology medicine.medical_specialty congenital hereditary and neonatal diseases and abnormalities Cystic fibrosis - pathology - physiopathology Cystic Fibrosis Congenic Cystic Fibrosis Transmembrane Conductance Regulator Disease Cystic fibrosis Severity of Illness Index Pulmonary fibrosis Pathogenesis Mice Fibrosis Parenchyma medicine Cystic fibrosis transmembrane conductance regulator - genetics - metabolism Animals Lung Ion transport Chloride channels Mice Knockout Lung mechanics Lung/microbiology - pathology - physiopathology - ultrastructure business.industry Obstructive airway disease General Medicine respiratory system medicine.disease respiratory tract diseases Respiratory Function Tests Electrophysiology Mice Inbred C57BL Pulmonary Alveoli Disease Models Animal Nasal Mucosa Immunology Chloride channel Female business Research Article |
| Zdroj: | Scopus-Elsevier |
| Popis: | 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 bee 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- knock-out 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. published_or_final_version |
| Databáze: | OpenAIRE |
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