mortality/aging
• life span is normal except that pregnancy-induced mortality is seen
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cardiovascular system
N |
• deletion had little affect on cardiomyocyte proliferation or viability
• mutants have a normal heart rate, interventricular septum thickness, and left ventricular posterior wall thickness
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• extensive fibrosis and myofibril disarray are evident by trichome staining in older mice
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• mutant hearts appear normal initially and become enlarged 5 weeks after birth
• increasing workload and stress results in manifestation of a latent defect in cardiac function
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• mutants have a severe contraction defect
• the percent fractional shortening and mean velocity of circumferential fiber shortening (indicators of systolic cardiac function) are severely reduced
• in isolated cardiomyocytes resting intracellular and diastolic Ca2+ is normal however, with increasing pacing frequencies a significant decrease in both Ca2+ transients and cell contraction are detected
• defects in Ca2+ relaxation kinetics and reduced Ca2+ transients would weaken the excitation-contraction coupling in response to increasing workload
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• both left ventricular end-diastolic dimensions and end-systolic dimensions are markedly increased 5 to 6 weeks after birth
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muscle
• mutant hearts appear normal initially and become enlarged 5 weeks after birth
• increasing workload and stress results in manifestation of a latent defect in cardiac function
|
• mutants have a severe contraction defect
• the percent fractional shortening and mean velocity of circumferential fiber shortening (indicators of systolic cardiac function) are severely reduced
• in isolated cardiomyocytes resting intracellular and diastolic Ca2+ is normal however, with increasing pacing frequencies a significant decrease in both Ca2+ transients and cell contraction are detected
• defects in Ca2+ relaxation kinetics and reduced Ca2+ transients would weaken the excitation-contraction coupling in response to increasing workload
|
• both left ventricular end-diastolic dimensions and end-systolic dimensions are markedly increased 5 to 6 weeks after birth
|
cellular
• extensive fibrosis and myofibril disarray are evident by trichome staining in older mice
|