mortality/aging
• maximal life span is shortened to 21 weeks
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cardiovascular system
• gradual accumulation of mitochondria with abnormal morphology in the myocardium from 5 weeks of age onwards
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• gradual increase in absolute and relative heart size with age
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• levels of assembled complexes containing mtDNA-encoded subunits are decreased from 5 weeks of age onward
• severe respiratory chain deficiency
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• severe mitochondrial cardiomyopathy develops from 5 weeks of age onwards
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cellular
• severe decrease of in organello translation in the hearts
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growth/size/body
• gradual increase in absolute and relative heart size with age
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• from 15 weeks of age
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muscle
• gradual accumulation of mitochondria with abnormal morphology in the myocardium from 5 weeks of age onwards
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• severe mitochondrial cardiomyopathy develops from 5 weeks of age onwards
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