Pitceathly, RDS;
Viscomi, C;
(2016)
Effects of ketosis in mitochondrial myopathy: potential benefits of a mitotoxic diet.
EMBO Molecular Medicine
, 8
(11)
pp. 1231-1233.
10.15252/emmm.201606933.
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Abstract
The field of mitochondrial medicine is rapidly transitioning from preclinical observation to clinical application. Translation of promising data obtained in mouse models is not always straightforward, however. Building on their own work showing that a ketogenic diet induces mitochondrial biogenesis and delays the onset of disease in the Deletor mouse, Ahola et al administered modified Atkins diet (mAD) to five patients with mitochondrial myopathy caused by mitochondrial DNA deletions (Ahola et al, 2016). Surprisingly, mAD did not induce mitochondrial biogenesis in patients, but rather triggered the progressive damage of muscle cells, particularly those with impaired respiratory chain activity (the ragged-red fibres). The subsequent extensive characterisation of the metabolic and molecular profile changes observed in patients and healthy controls provides a significant advance towards understanding the feasibility of dietary modification as a treatment strategy for mitochondrial diseases.
| Type: | Article |
|---|---|
| Title: | Effects of ketosis in mitochondrial myopathy: potential benefits of a mitotoxic diet |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.15252/emmm.201606933 |
| Publisher version: | http://doi.org/10.15252/emmm.201606933 |
| Language: | English |
| Additional information: | © 2016 The Authors. Published under the terms of the CC BY 4.0 license. This is an open access article under the terms of the Creative Commons Attribution 4.0 License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited. |
| Keywords: | Science & Technology, Life Sciences & Biomedicine, Medicine, Research & Experimental, Research & Experimental Medicine, KETOGENIC DIET, DISEASE |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Department of Neuromuscular Diseases |
| URI: | https://discovery.ucl.ac.uk/id/eprint/1524782 |
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