mortality/aging
• die within 10 minutes of birth
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respiratory system
• present in neonates, particularly around the large blood vessels; however no signs of hemorrhage are seen any where else in the body
• rupture of the vascular smooth muscle wall and herniation of the endothelial lining is seen in several vessel that show hemorrhage
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• large vessels appear dilated, the smooth muscle layer appears hypertrophic, and at E18.5 branching of the smaller vessels is reduced
• some large vessels have less smooth muscle and in neonates increased TUNEL staining is seen in vascular smooth muscle cells but not in bronchial smooth muscle
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• at E12.5 proliferation of the distal mesenchyme is reduced by about 2/3 compared to wild-type mice, but by E14.5 a difference in proliferation is no longer detected
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• prominent thinning of the distal mesenchyme at E12.5
• distal mesenchyme is sometimes only one or two cell layers thick
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• at E18.5 almost no alveolar epithelial type 1 cells are detected although type 2 cells and surfactant are present
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• seen at E12.5 with prominent thinning of the distal mesenchyme
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• gasp for breath and do not show evidence of air in the distal airways
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cardiovascular system
• large vessels appear dilated, the smooth muscle layer appears hypertrophic, and at E18.5 branching of the smaller vessels is reduced
• some large vessels have less smooth muscle and in neonates increased TUNEL staining is seen in vascular smooth muscle cells but not in bronchial smooth muscle
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• present in neonates, particularly around the large blood vessels; however no signs of hemorrhage are seen any where else in the body
• rupture of the vascular smooth muscle wall and herniation of the endothelial lining is seen in several vessel that show hemorrhage
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cellular
• at E12.5 proliferation of the distal mesenchyme is reduced by about 2/3 compared to wild-type mice, but by E14.5 a difference in proliferation is no longer detected
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