mortality/aging
• mice die at 19 weeks
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cardiovascular system
• mice show signs of progressive heart enlargement from age of 8 weeks
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cellular
• severe depletion of mitochondrial DNA (mtDNA) is observed in heart-specific mutants; this results in decrease of mtDNA-encoded transcripts and mtDNA-encoded proteins like Cox1 and Cox2
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• in heart tissue, levels respiratory complexes and superassemblies containing mtDNA-encoded subunits are decreased complared to an exclusively nucleus-encoded complex indicating that the mutation results in defective respiratory chain assembly in heart mitochondria
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growth/size/body
• mice show signs of progressive heart enlargement from age of 8 weeks
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