mortality/aging
• due to respiratory failure
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respiratory system
• impaired secondary septation
• bronchial myogenesis and alveolar myofibroblast development are impaired
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• branching morphogenesis is disrupted during the pseudoglandular stage
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• at P9, mice exhibit abnormal alveolization with dilated distal air sacs and reduced number of terminal airspaces
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• at E13.5 and E14.5, distal epithelial buds are dilated with reduced branching compared to in wild-type mice
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• with few secondary septations at 3 weeks of age
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• in some mice
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growth/size/body
• in surviving mice
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cellular
• mouse embryonic fibroblasts exhibit impaired development into myofibroblasts
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homeostasis/metabolism