S. B. Vafai and V. K. Mootha, Mitochondrial disorders as windows into an ancient organelle, Nature, vol.399, pp.374-383, 2012.

W. J. Koopman, P. H. Willems, and J. A. Smeitink, Monogenic mitochondrial disorders, N Engl J Med, vol.366, pp.401-1132, 2012.

D. Donato and S. , Disorders related to mitochondrial membranes: pathology of the respiratory chain and 403 neurodegeneration, J Inherit Metab Dis, vol.23, pp.247-263, 2000.

J. R. Roede, Y. M. Go, and D. P. Jones, Redox equivalents and mitochondrial bioenergetics, Methods Mol Biol, vol.405, pp.249-280, 2012.

A. Munnich, A. Rotig, D. Chretien, V. Cormier, T. Bourgeron et al.,

, Clinical presentation of mitochondrial disorders in childhood, J Inherit Metab Dis, vol.19, pp.408-521, 1996.

D. R. Thorburn, Mitochondrial disorders: prevalence, myths and advances, J Inherit Metab Dis, vol.410, pp.349-362, 2004.

M. Schiff, P. Benit, A. Coulibaly, S. Loublier, R. El-khoury et al., Mitochondrial response to controlled 412 nutrition in health and disease, Nutr Rev, vol.69, pp.65-75, 2011.

M. Schiff, P. Benit, H. T. Jacobs, J. Vockley, and R. P. , Therapies in inborn errors of oxidative metabolism

, Trends Endocrinol Metab, vol.23, pp.488-495, 2012.

S. Passarella, A. A. Valenti, D. De, and B. L. , The role of mitochondrial transport in energy metabolism, 416 Mitochondrion, vol.2, pp.319-343, 2003.

D. C. Wallace, W. Fan, and V. Procaccio, Mitochondrial energetics and therapeutics, Annu Rev Pathol, vol.418, pp.297-348, 2010.

G. J. , The role of nutrition in mitochondrial an metabolic diseases. A primary care physician's 420 guide, pp.15-16, 2000.

E. N. Mokhova and L. S. Khailova, Involvement of mitochondrial inner membrane anion carriers in the 422 uncoupling effect of fatty acids, Biochemistry (Mosc), vol.70, pp.159-63, 2005.

R. B. Doctor, R. Bacallao, and L. J. Mandel, Method for recovering ATP content and mitochondrial function 424 after chemical anoxia in renal cell cultures, Am J Physiol, vol.266, pp.1803-1811, 1994.

S. Santra, R. W. Gilkerson, M. Davidson, and E. A. Schon, Ketogenic treatment reduces deleted mitochondrial 426

, DNAs in cultured human cells, Ann Neurol, vol.56, pp.662-669, 2004.

M. Schiff, P. Benit, R. El-khoury, D. Schlemmer, J. F. Benoist et al., Mouse studies to shape clinical 428 trials for mitochondrial diseases: high fat diet in Harlequin mice, PLoS One, vol.6, 2011.

M. J. Roef, K. De-meer, D. J. Reijngoud, H. W. Straver, M. De-barse et al., Triacylglycerol 431 infusion improves exercise endurance in patients with mitochondrial myopathy due to 432 complex I deficiency, Am J Clin Nutr, vol.75, pp.237-244, 2002.

M. J. Roef, K. De-meer, D. J. Reijngoud, H. W. Straver, M. De-barse et al., Triacylglycerol 434 infusion does not improve hyperlactemia in resting patients with mitochondrial myopathy

M. J. Roef, D. J. Reijngoud, J. A. Jeneson, R. Berger, and M. K. De, Resting oxygen consumption and in vivo ADP are 437 increased in myopathy due to complex I deficiency, Neurology, vol.58, pp.1088-1093, 2002.

J. Panetta, L. J. Smith, and A. Boneh, Effect of high-dose vitamins, coenzyme Q and high-fat diet in paediatric 439 patients with mitochondrial diseases, J Inherit Metab Dis, vol.27, pp.487-498, 2004.

F. P. Bernier, A. Boneh, X. Dennett, C. W. Chow, M. A. Cleary et al., Diagnostic criteria for 441 respiratory chain disorders in adults and children, Neurology, vol.59, pp.1406-1411, 2002.

K. F. Schulz, D. G. Altman, D. Moher, and . Consort, statement: Updated guidelines for reporting parallel 443 group randomised trials, J Pharmacol Pharmacother, vol.1, pp.100-107, 2010.

A. Martin, Apports nutritionnels conseillés pour la population française, TEC et DOC Lavoisier, 2000.

A. Z. Belko, T. F. Barbieri, and E. C. Wong, Effect of energy and protein intake and exercise intensity on the 447 thermic effect of food, Am J Clin Nutr, vol.43, pp.863-869, 1986.

J. A. Barnard, L. C. Tapsell, P. S. Davies, V. L. Brenninger, and L. H. Storlien, Relationship of high energy expenditure 449 and variation in dietary intake with reporting accuracy on 7 day food records and diet 450 histories in a group of healthy adult volunteers, Eur J Clin Nutr, vol.56, pp.358-367, 2002.

, Informatic Center for Quality of foods, Pictures of different meals, pp.1-63, 1993.

A. A. Welch, A. Mctaggart, A. A. Mulligan, R. Luben, N. Walker et al., , p.453

, Epidemiological Research) -a new data-entry program for nutritional 454 analysis in the EPIC-Norfolk cohort and the 7-day diary method, Public Health Nutr, vol.4, pp.455-1253, 2001.

F. W. Chattaway, F. C. Happold, and A. M. Happold, Nutrition of school-children in Leeds, winter, 1943, and 457 summer, 1944, Br Med J, vol.1, p.429, 1946.

F. E. Dougherty, Metabolic testing in mitochondrial disease, Semin Neurol, vol.21, pp.303-308

E. Blond, C. Maitrepierre, S. Normand, M. Sothier, and H. Roth, A new indirect calorimeter is accurate and 460 reliable for measuring basal enerby expenditure, thermic effect of food and substrate 461 oxidation in obese and healthy subjects. e-jounal of, Clinical nutrition, 2010.

J. B. Weir, New methods for calculating metabolic rate with special reference to protein metabolism, J 463 Physiol, vol.109, pp.1-9, 1949.

M. Dabbech, A. Boulier, M. Apfelbaum, and A. R. , Thermic effect of meal and fat mass in lean and obese 465 men, Nutrition Research, vol.16, pp.1133-1141, 1996.

M. I. Goran, P. Driscoll, R. Johnson, T. R. Nagy, and G. Hunter, Cross-calibration of body-composition techniques 467 against dual-energy X-ray absorptiometry in young children, Am J Clin Nutr, vol.63, pp.299-305, 1996.

M. Putt and V. M. Chinchilli, A mixed effects model for the analysis of repeated measures cross-over 469 studies, Stat Med, vol.18, pp.3037-3058, 1999.

X. Chen and L. Wei, A comparison of recent methods for the analysis of small-sample cross-over studies

, Stat Med, vol.22, pp.2821-2833, 2003.

P. Benit, R. El-khoury, M. Schiff, A. Sainsard-chanet, and R. P. , Genetic background influences