Analysis Tools
cardiovascular system
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• at 42 days, the space between each elastin fiber is significantly reduced in mutant aortae
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• at 42 days, the space occupied by smooth muscle cells in the vessel wall is significantly reduced
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• adult homozygotes exhibit calcification and degeneration of the cardiac muscle
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• at 42 days, myocardial fibers appear to be fragile and prone to breakage upon mechanical stress
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• at 42 days, homozygotes exhibit necrosis on the surface of the heart
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• at 42 days, homozygotes display fibrosis at the surface of the right ventricle and in the septum
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• at ~30 days, mutant hearts display white spots corresponding to calcification, as confirmed by Kossa's method
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hemorrhage
(
J:32928
)
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• at 42 days, homozygotes exhibit hemorrhaging on the surface of the heart
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muscle
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• at 42 days, the space occupied by smooth muscle cells in the vessel wall is significantly reduced
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• adult homozygotes exhibit calcification and degeneration of the cardiac muscle
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• at 42 days, myocardial fibers appear to be fragile and prone to breakage upon mechanical stress
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• at 42 days, homozygotes exhibit necrosis on the surface of the heart
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• mitochondria lose association with Z-discs
• mitochondria found in focal aggregates in sarcoplasm and subsarcolemmal regions
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• atrophic fibers seen at 1 year
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• hypertrophic fibers seen at 1 year
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• centrally nucleated fibers in the soleus at 1 year
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• marked reduction in total number of fibers
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• numerous necrotic fibers in the soleus at 1 year
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• at 42 days, mutant diaphragms exhibit abnormal sarcomeres with no clear demarcation of I and A bands and disintegrated fibers with sparse filaments
• individual muscle fibers are commonly misaligned and disoriented
• in contrast, other muscles (e.g. limb muscles) exhibit normal sarcomere alignment up to 12 months of age
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• homozygotes display a thinner, transparent diaphragm relative to wild-type mice
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