respiratory system
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• impaired alveolar formation secondary to reduced airspace epithelial proliferation
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• at P5, proliferation of airway and alveolar epithelial cells in the developing airspace and airway of the mutant lung is reduced by 27% relative to wild-type controls
• however, lung epithelial differentiation is normal
• no differences in apoptosis are observed in the lung parenchyma
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• at P5 and P10, the number of mutant pulmonary NEBs is significantly higher than that of wild-type controls
• however, the number of mutant pulmonary NEBs is fully normalized by 6 weeks of age
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• at P5 and P10, distal airspace caliber is significantly increased relative to wild-type controls, as determined by mean linear intercept parameters
• no further increase in distal airspace caliber is noted after P10
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endocrine/exocrine glands
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• at P5 and P10, the number of mutant solitary PNECs is strikingly reduced relative to that in wild-type controls
• however, the number of mutant PNECs is fully normalized by 6 weeks of age
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nervous system
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• at P5 and P10, the number of mutant solitary PNECs is strikingly reduced relative to that in wild-type controls
• however, the number of mutant PNECs is fully normalized by 6 weeks of age
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