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• W2000775246 endingPage "1207" @default.
• W2000775246 startingPage "1198" @default.
• W2000775246 abstract "Mitochondria are essential organelles within the cell where most of the energy production occurs by the oxidative phosphorylation system (OXPHOS). Critical components of the OXPHOS are encoded by the mitochondrial DNA (mtDNA) and therefore, mutations involving this genome can be deleterious to the cell. Post-mitotic tissues, such as muscle and brain, are most sensitive to mtDNA changes, due to their high energy requirements and non-proliferative status. It has been proposed that mtDNA biological features and location make it vulnerable to mutations, which accumulate over time. However, although the role of mtDNA damage has been conclusively connected to neuronal impairment in mitochondrial diseases, its role in age-related neurodegenerative diseases remains speculative. Here we review the pathophysiology of mtDNA mutations leading to neurodegeneration and discuss the insights obtained by studying mouse models of mtDNA dysfunction." @default.
• W2000775246 created "2016-06-24" @default.
• W2000775246 creator A5014131126 @default.
• W2000775246 creator A5060561492 @default.
• W2000775246 date "2014-08-01" @default.
• W2000775246 modified "2023-10-02" @default.
• W2000775246 title "Mitochondrial genome changes and neurodegenerative diseases" @default.
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