mortality/aging
• mice die between 2 and 3 weeks of age likely from airway obstruction and asphyxiation
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respiratory system
• mice exhibit large dense patches consisting of infiltrating neutrophils that is not secondary to bacterial infection unlike control mice
• however, treatment with amiloride reduces inflammation
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• mice exhibit decreased lung wet/dry ratio compared with control mice
• however, treatment with amiloride improves lung defects
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• mice exhibit cystic fibrosis-like defects unlike control mice
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• mice exhibit increased nonciliated mucus secreting (mostly goblet) cells in the trachea unlike control mice
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• mice exhibit increased nonciliated mucus secreting (mostly goblet) cells in the trachea unlike control mice
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• airway periciliary liquid layer in the trachea is decreased in height compared to in control mice
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immune system
• mice exhibit large dense patches consisting of infiltrating neutrophils that is not secondary to bacterial infection unlike control mice
• however, treatment with amiloride reduces inflammation
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