mortality/aging
• die within the first year of life
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cardiovascular system
• cardiomyocyte length is increased and they exhibit enlarged nuclei with a significantly higher rate of polyploidy at 3 months of age
• dilation of tubular membrane system in myocytes with normal myofibrils and mitochondria
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• number of intercalated disks per unit area of ventricular muscle is reduced
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• number of nuclei per unit area of ventricular muscle is reduced
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• exhibit higher heart-to-body weight ratios than wildtype at 3 months of age
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• hearts are enlarged, however observe no differences in cardiomyocyte cell number, proliferation or apoptosis
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• dilation evident by 3 months of age but is not seen in neonates
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• evident by 3 months of age
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• dilation evident by 3 months of age but is not seen in neonates
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• retrograde perfusion of the left ventricle in isolated hearts shows a reduction of the maximal left ventricular developed pressure, demonstrating depressed myocardial contractility
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• programmed intracardiac stimulation shows increased susceptibility to ventricular tachycardia
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• ECG at 2-3 months of age shows widened QRS complex and Q-Tc intervals, indicating delayed conduction
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• ECG at 2-3 months of age shows widened QRS complex
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• 12.5% prolongation in Q-Tc intervals
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muscle
• cardiomyocyte length is increased and they exhibit enlarged nuclei with a significantly higher rate of polyploidy at 3 months of age
• dilation of tubular membrane system in myocytes with normal myofibrils and mitochondria
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• number of intercalated disks per unit area of ventricular muscle is reduced
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• number of nuclei per unit area of ventricular muscle is reduced
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• retrograde perfusion of the left ventricle in isolated hearts shows a reduction of the maximal left ventricular developed pressure, demonstrating depressed myocardial contractility
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growth/size/body
• exhibit higher heart-to-body weight ratios than wildtype at 3 months of age
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• hearts are enlarged, however observe no differences in cardiomyocyte cell number, proliferation or apoptosis
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