Analysis Tools
cardiovascular system
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• vessels with multiple elastin fiber (MEF - vessels surrounded fully by internal and external elastic lamina) are seen more frequently in mutant lungs at P8
• vessels with a single elastin fiber (SEF - vessels surrounded by a single elastic lamina) accordingly decrease in mutant lungs at P8 compared to wild-type
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• extreme disarrangement of striated muscle fibers is noted
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• degeneration is indicated by disarrangement of muscle fibers
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• slight enlargement is noted at E17.5 relative to wild-type
• at P8 and P15, enlargement becomes prominent
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• slight enlargement is noted at E17.5 relative to wild-type; differences measured in RV dimensions are statistically significant
• at P8 and P15, enlargement becomes prominent
• however, the RV wall does not display any apparent hypertrophy
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• right ventricular blood pressure in mutant hearts is significantly lower than in wild-type mice
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respiratory system
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• impaired lung remodeling at the late embryonic stage, leading to right ventricular enlargement in the adult stage
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• in lungs from P2 to P15, walls of secondary septa have subdivided alveoli, whereas wild-type lungs have incompletely partitioned smooth channels and saccules, indicating that lung alveolar development progresses faster in mutants
• defective space increase in interalveolar septa and hyperplasia of endothelial cells is seen during alveolar formation
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• reduction in mesenchyme is observed at E17.5 in embryonic lungs
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• incomplete alveolar spaces are occasionally observed in P15 and adult mutant lungs
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muscle
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• extreme disarrangement of striated muscle fibers is noted
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• degeneration is indicated by disarrangement of muscle fibers
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