[HTML][HTML] Airway hyperresponsiveness in FVB/N delta F508 cystic fibrosis transmembrane conductance regulator mice

M Bazett, CK Haston - Journal of Cystic Fibrosis, 2014 - Elsevier
M Bazett, CK Haston
Journal of Cystic Fibrosis, 2014Elsevier
Background Airway hyperresponsiveness is a feature of clinical CF lung disease. In this
study, we investigated whether the FVB/N ΔF508 CFTR mouse model has altered airway
mechanics. Methods Mechanics were measured in 12–14 week old FVB/N Cftr tm1Eur
(ΔF508) mice and wildtype littermates using the FlexiVent small animal ventilator. Lung
disease was assayed by immunohistochemistry, histology and bronchoalveolar lavage
analysis. Results Cftr tm1Eur mice presented with increased airway resistance, compared to …
Background
Airway hyperresponsiveness is a feature of clinical CF lung disease. In this study, we investigated whether the FVB/N ΔF508 CFTR mouse model has altered airway mechanics.
Methods
Mechanics were measured in 12–14 week old FVB/N Cftrtm1Eur (ΔF508) mice and wildtype littermates using the FlexiVent small animal ventilator. Lung disease was assayed by immunohistochemistry, histology and bronchoalveolar lavage analysis.
Results
Cftrtm1Eur mice presented with increased airway resistance, compared to wildtype littermates, in response to methacholine challenge. No differences in bronchoalveolar cell number or differential, or in tissue lymphocyte, goblet cell or smooth muscle actin levels were evident in mice grouped by Cftr genotype. The bronchoalveolar lavage of Cftrtm1Eur mice included significantly increased levels of interleukin 12(p40) and CXCL1 compared to controls.
Conclusion
We conclude that the pulmonary phenotype of Cftrtm1Eur mice includes airway hyperresponsiveness in the absence of overt lung inflammation or airway remodeling.
Elsevier