mortality/aging
• all mice die before 4 months of age
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• some pups show delayed growth after P7 and die before weaning
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growth/size/body
• hearts are larger at P10, P21, and 8 weeks of age
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• some pups show delayed growth after P7
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cardiovascular system
• localization of desmin in intercalated discs is reduced and pattern of desmin localization is perturbed
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• hearts are larger at P10, P21, and 8 weeks of age
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• thinner anterior and posterior ventricle walls
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• broad myocardium interstitial fibrosis
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• chamber dilation
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• in 8 week old hearts
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• 8 week old hearts show reduced fractional shortening and decreased chamber contraction ratio
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• echocardiography of 8 week old hearts indicates dilated left ventricles, thinner anterior and posterior ventricle walls, decreased chamber contraction ratio, increased aortic time-velocity integral (the distance traveled by a volume of blood during a time interval), reduced fractional shortening and cardiac output
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• mice exhibit shortened conduction time through the atrioventricular node (PR interval)
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• P wave height, which indicates atrial depolarization, is reduced
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• some mice survive after weaning without obvious defects, but at 4-6 weeks exhibit symptoms of heart failure, including weight loss, reduced activity level, hunched back and labored breath
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muscle
• localization of desmin in intercalated discs is reduced and pattern of desmin localization is perturbed
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• 8 week old hearts show reduced fractional shortening and decreased chamber contraction ratio
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• high level of apoptosis in the myocardium
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• sarcomere length in cardiomyocytes is shorter
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cellular
• broad myocardium interstitial fibrosis
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• high level of apoptosis in the myocardium
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