2010
DOI: 10.1093/hmg/ddq076
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Ketogenic diet slows down mitochondrial myopathy progression in mice

Abstract: Mitochondrial dysfunction is a major cause of neurodegenerative and neuromuscular diseases of adult age and of multisystem disorders of childhood. However, no effective treatment exists for these progressive disorders. Cell culture studies suggested that ketogenic diet (KD), with low glucose and high fat content, could select against cells or mitochondria with mutant mitochondrial DNA (mtDNA), but proper patient trials are still lacking. We studied here the transgenic Deletor mouse, a disease model for progres… Show more

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Cited by 169 publications
(142 citation statements)
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“…A perceived improvement in mitochondrial function is not always related to an obvious decrease in the proportion of mutant mtDNA, which makes it difficult to assess a change in mtDNA heteroplasmy [40]. Because an obvious decrease in the mtDNA mutation load in the rescued MERRF cybrid cells was not observed in our study, it was necessary to evaluate the mitochondrial homeostasis regulatory mechanisms.…”
Section: Discussionmentioning
confidence: 95%
“…A perceived improvement in mitochondrial function is not always related to an obvious decrease in the proportion of mutant mtDNA, which makes it difficult to assess a change in mtDNA heteroplasmy [40]. Because an obvious decrease in the mtDNA mutation load in the rescued MERRF cybrid cells was not observed in our study, it was necessary to evaluate the mitochondrial homeostasis regulatory mechanisms.…”
Section: Discussionmentioning
confidence: 95%
“…Ketogenic diet treatment of mice with a defective twinkle helicase, which accumulate deleted mtDNA molecules, ameliorated the OXPHOS deficiency and improved the phenotype of the mitochondrial myopathy associated with the loss of twinkle function. However, the ketogenic diet did not affect mtDNA quantity and quality [Ahola-Erkkila et al, 2010]. These results suggest that the ketogenic diet might be useful for the treatment of mitochondrial myopathies, but the mechanisms of this therapeutic effect still remains to be elucidated.…”
Section: Ketogenic Dietmentioning
confidence: 78%
“…These phenomena could contribute to explain the anticonvulsant effects of KD. In a preclinical trial on the deletor mouse, KD slowed the progression of mitochondrial myopathy [85]. However, other reports showed that KD can have the opposite effect, and worsens the mitochondrial defect in vivo, for instance in the Mterf2 −/− [86], or the Mpv17 -/− mouse models [87].…”
Section: Dietary Manipulationsmentioning
confidence: 98%