respiratory system
• at E10.5 and E11.5, the primary branching of the right lung is impaired; however, the left lung develops normally
• at E14.5, but not at E11.5, mutant lungs show a 40% and 25% reduction in the number proliferating (BrdU+) cells in the mesenchymal and epithelial compartments, respectively
• at E18.5, mutant terminal air sacs are smaller and lined by thicker interstitial layers
• however, mutant epithelial cells can undergo terminal differentiation
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• at E14.5, but not at E11.5, mutant lungs show a 40% reduction in the number proliferating (BrdU+) cells in the mesenchymal compartment
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• at E18.5, mutant terminal air sacs are smaller and lined by thicker interstitial layers
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• at E18.5, mutant interstitial mesenchymal cell layers are thicker than normal
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• at E18.5, the density of surfactant protein C (a marker for mature alveolar type II cells)-positive cells is increased relative to that in wild-type controls
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small lung
(
J:49603
)
• at E10.5 and E11.5, mutant lungs are significantly smaller than wild-type
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• at E13.5 and E18.5, the wet weight of the mutant left lung is ~60% and 50% of that in wild-type and heterozygous controls, respectively
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• lung hypoplasia is likely due to reduced cell proliferation at later stages of lung development
• no differences in apoptotic cell death are observed relative to wild-type controls
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• at E10.5 and E11.5, a one-lobed right lung is observed, unlike the four-lobed right lung seen in wild-type embryos
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• at E18.5, the cartilaginous rings of the mutant trachea are smaller and split in some cases
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• at E18.5, the mutant trachea is hypoplastic
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• at E18.5
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digestive/alimentary system
• the mutant esophagus fails to develop a smooth muscle layer
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• homozygotes display foregut malformations, including stenosis of the esophagus and trachea, lung hypoplasia and lung lobulation defects
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• at E18.5, the mutant esophagus lacks smooth muscle
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• at E18.5
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• at E18.5, the mutant esophagus is hypoplastic with a very small lumen that lacks the typical folded structure seen in wild-type controls
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nervous system
• expression of ventral neural tube patterning markers is altered compared to in wild-type mice
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embryo
• expression of ventral neural tube patterning markers is altered compared to in wild-type mice
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muscle
• at E18.5, the mutant esophagus lacks smooth muscle
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skeleton
• at E18.5, the cartilaginous rings of the mutant trachea are smaller and split in some cases
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cellular
• at E14.5, but not at E11.5, mutant lungs show a 40% reduction in the number proliferating (BrdU+) cells in the mesenchymal compartment
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