mortality/aging
• 50% mortality at 21 weeks of age
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cardiovascular system
• extensive thinning of the ventricular walls, especially in the right ventricle
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• stretches of interstitial fibrosis in the ventricles and atria at 6 weeks of age
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• mice develop a severely dilated cardiomyopathy after 6 weeks of age
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• cardiac function is decreased, with a reduction of ejection fraction from 70% to 54% and a 30% decrease in fractional shortening at 6 weeks of age
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• echocardiography at 6 weeks of age shows increased left ventricular internal diameter in systole and diastole
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cellular
• stretches of interstitial fibrosis in the ventricles and atria at 6 weeks of age
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• gene expression analysis indicates disturbed mitochondrial function and downregulation of mitochondrial oxidative phosphorylation complexes III and IV at the protein level, suggesting that cardiomyocytes have impaired oxidative capacity
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muscle
• mice develop a severely dilated cardiomyopathy after 6 weeks of age
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• cardiac function is decreased, with a reduction of ejection fraction from 70% to 54% and a 30% decrease in fractional shortening at 6 weeks of age
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