FVB/N ΔF508囊性纤维化跨膜传导调节因子小鼠的气道高反应性
Airway hyperresponsiveness in FVB/N delta F508 cystic fibrosis transmembrane conductance regulator mice.
作者信息
Bazett Mark, Haston Christina K
机构信息
Department of Human Genetics, McGill University, 3626 St. Urbain, Montreal, Qc H2X 2P2, Canada; Department of Medicine, McGill University, 3626 St. Urbain, Montreal, Qc H2X 2P2, Canada; The Meakins-Christie Laboratories, McGill University, 3626 St. Urbain, Montreal, Qc H2X 2P2, Canada.
Department of Human Genetics, McGill University, 3626 St. Urbain, Montreal, Qc H2X 2P2, Canada; Department of Medicine, McGill University, 3626 St. Urbain, Montreal, Qc H2X 2P2, Canada; The Meakins-Christie Laboratories, McGill University, 3626 St. Urbain, Montreal, Qc H2X 2P2, Canada.
出版信息
J Cyst Fibros. 2014 Jul;13(4):378-83. doi: 10.1016/j.jcf.2013.11.010. Epub 2013 Dec 24.
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 12-14week 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 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 Cftr(tm1Eur) mice included significantly increased levels of interleukin 12(p40) and CXCL1 compared to controls.
CONCLUSION
We conclude that the pulmonary phenotype of Cftr(tm1Eur) mice includes airway hyperresponsiveness in the absence of overt lung inflammation or airway remodeling.
背景
气道高反应性是临床囊性纤维化（CF）肺部疾病的一个特征。在本研究中，我们调查了FVB/N ΔF508 CFTR小鼠模型是否存在气道力学改变。
方法
使用FlexiVent小动物呼吸机对12 - 14周龄的FVB/N Cftr(tm1Eur)（ΔF508）小鼠和野生型同窝小鼠的力学进行测量。通过免疫组织化学、组织学和支气管肺泡灌洗分析来检测肺部疾病。
结果
与野生型同窝小鼠相比，Cftr(tm1Eur)小鼠在乙酰甲胆碱激发试验中气道阻力增加。按Cftr基因型分组的小鼠在支气管肺泡细胞数量或分类、或组织淋巴细胞、杯状细胞或平滑肌肌动蛋白水平上没有明显差异。与对照组相比，Cftr(tm1Eur)小鼠的支气管肺泡灌洗液中白细胞介素12（p40）和CXCL1水平显著升高。
结论
我们得出结论，Cftr(tm1Eur)小鼠的肺部表型包括在无明显肺部炎症或气道重塑情况下的气道高反应性。
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