E. J. Tucker, A. G. Compton, S. E. Calvo, and D. R. Thorburn, The molecular basis of human complex I deficiency, IUBMB Life, vol.63, pp.669-677, 2011.

F. Distelmaier, W. J. Koopman, L. P. Van-den-heuvel, R. J. Rodenburg, E. Mayatepek et al., Mitochondrial complex I deficiency: from organelle dysfunction to clinical disease, Brain: J. Neurol, vol.132, pp.833-842, 2009.

S. Papa and D. De-rasmo, Complex I deficiencies in neurological disorders, Trends Mol. Med, vol.19, pp.61-69, 2013.

T. B. Haack, B. Haberberger, E. M. Frisch, T. Wieland, A. Iuso et al., Molecular diagnosis in mitochondrial complex I deficiency using exome sequencing, J. Med. Genet, vol.49, pp.277-283, 2012.

S. Verkaart, W. J. Koopman, S. E. Van-emst-de-vries, L. G. Nijtmans, L. W. Van-den-heuvel et al., Superoxide production is inversely related to complex I activity in inherited complex I deficiency, Biochim. Biophys. Acta, vol.1772, pp.373-381, 2007.
URL : https://hal.archives-ouvertes.fr/hal-00501647

P. Roestenberg, G. R. Manjeri, F. Valsecchi, J. A. Smeitink, P. H. Willems et al., Pharmacological targeting of mitochondrial complex I deficiency: the cellular level and beyond, Mitochondrion, vol.12, pp.57-65, 2012.

F. Valsecchi, W. J. Koopman, G. R. Manjeri, R. J. Rodenburg, J. A. Smeitink et al., Complex I disorders: causes, mechanisms, and development of treatment strategies at the cellular level, Dev. Disabil. Res. Rev, vol.16, pp.175-182, 2010.

J. Tome-carneiro, M. Larrosa, A. Gonzalez-sarrias, F. A. Tomas-barberan, M. T. Garcia-conesa et al., Resveratrol and clinical trials: the crossroad from in vitro studies to human evidence, Curr. Pharm. Des, vol.19, pp.6064-6093, 2013.

S. S. Kulkarni and C. Canto, The molecular targets of resveratrol, Biochim. Biophys. Acta, vol.1852, pp.1114-1123, 2015.

A. Costa, C. L. Bachelier, L. Mathieu, A. Rotig, A. Boneh et al., Beneficial effects of resveratrol on respiratory chain defects in patients' fibroblasts involve estrogen receptor and estrogen-related receptor alpha signaling, Hum. Mol. Genet, vol.23, pp.2106-2119, 2014.
URL : https://hal.archives-ouvertes.fr/inserm-02894840

S. Bastianetto, C. Menard, and R. Quirion, Neuroprotective action of resveratrol, Biochim. Biophys. Acta, vol.1852, pp.1195-1201, 2015.

A. Giralt and F. Villarroya, SIRT3, a pivotal actor in mitochondrial functions: metabolism, cell death and aging, Biochem. J, vol.444, pp.1-10, 2012.

Y. Chen, J. Zhang, Y. Lin, Q. Lei, K. L. Guan et al., Tumour suppressor SIRT3 deacetylates and activates manganese superoxide dismutase to scavenge ROS, EMBO Rep, vol.12, pp.534-541, 2011.

X. Qiu, K. Brown, M. D. Hirschey, E. Verdin, and D. Chen, Calorie restriction reduces oxidative stress by SIRT3-mediated SOD2 activation, Cell Metab, vol.12, pp.662-667, 2010.

R. Tao, M. C. Coleman, J. D. Pennington, O. Ozden, S. H. Park et al., Sirt3-mediated deacetylation of evolutionarily conserved lysine 122 regulates MnSOD activity in response to stress, Mol. Cell, vol.40, pp.893-904, 2010.

C. Aguer, D. Gambarotta, R. J. Mailloux, C. Moffat, R. Dent et al., Galactose enhances oxidative metabolism and reveals mitochondrial dysfunction in human primary muscle cells, PloS One, vol.6, p.28536, 2011.

A. Golubitzky, P. Dan, S. Weissman, G. Link, J. D. Wikstrom et al., Screening for active small molecules in mitochondrial complex I deficient patient's fibroblasts, reveals AICAR as the most beneficial compound, PloS One, vol.6, p.26883, 2011.

A. J. Janssen, F. J. Trijbels, R. C. Sengers, J. A. Smeitink, L. P. Van-den-heuvel et al., Spectrophotometric assay for complex I of the respiratory chain in tissue samples and cultured fibroblasts, Clin. Chem, vol.53, pp.729-734, 2007.

F. Paoletti and A. Mocali, Determination of superoxide dismutase activity by purely chemical system based on NAD(P)H oxidation, Methods Enzymol, vol.186, pp.209-220, 1990.

F. Caruso, J. Tanski, A. Villegas-estrada, and M. Rossi, Structural basis for antioxidant activity of trans-resveratrol: ab initio calculations and crystal and molecular structure, J. Agric. Food Chem, vol.52, pp.7279-7285, 2004.

M. Fukui, H. J. Choi, and B. T. Zhu, Mechanism for the protective effect of resveratrol against oxidative stress-induced neuronal death, Free Radic, Biol. Med, vol.49, pp.800-813, 2010.

E. L. Robb and J. A. Stuart, Resveratrol interacts with estrogen receptor-beta to inhibit cell replicative growth and enhance stress resistance by upregulating mitochondrial superoxide dismutase, Free Radic, Biol. Med, vol.50, pp.821-831, 2011.

E. L. Robb and J. A. Stuart, The stilbenes resveratrol, pterostilbene and piceid affect growth and stress resistance in mammalian cells via a mechanism requiring estrogen receptor beta and the induction of Mn-superoxide dismutase, Phytochemistry, vol.98, pp.164-173, 2014.

E. L. Robb, L. Winkelmolen, N. Visanji, J. Brotchie, and J. A. Stuart, Dietary resveratrol administration increases MnSOD expression and activity in mouse brain, Biochem. Biophys. Res. Commun, vol.372, pp.254-259, 2008.

G. Deblois, J. A. Hall, M. C. Perry, J. Laganiere, M. Ghahremani et al., Genome-wide identification of direct target genes implicates estrogen-related receptor alpha as a determinant of breast cancer heterogeneity, Cancer Res, vol.69, pp.6149-6157, 2009.

L. R. Stein and S. Imai, The dynamic regulation of NAD metabolism in mitochondria, Trends Endocrinol. Metab, vol.23, pp.420-428, 2012.

R. Cerutti, E. Pirinen, C. Lamperti, S. Marchet, A. A. Sauve et al., NAD(þ )-dependent activation of Sirt1 corrects the phenotype in a mouse model of mitochondrial disease, Cell Metab, vol.19, pp.1042-1049, 2014.
URL : https://hal.archives-ouvertes.fr/hal-02369963

R. Felici, A. Lapucci, L. Cavone, S. Pratesi, R. Berlinguer-palmini et al., Pharmacological NAD-boosting strategies improve mitochondrial homeostasis in human complex I-Mutant fibroblasts, Mol. Pharmacol, vol.87, pp.965-971, 2015.

N. A. Khan, M. Auranen, I. Paetau, E. Pirinen, L. Euro et al., Effective treatment of mitochondrial myopathy by nicotinamide riboside, a vitamin B3, EBO Mol. Med, vol.6, pp.721-731, 2014.

C. Canto and K. J. Menzies, NAD( þ) metabolism and the control of energy homeostasis: a balancing act between mitochondria and the nucleus, Cell Metab, vol.22, pp.31-53, 2015.

L. Mouchiroud and R. H. Houtkooper, NAD(þ ) metabolism: a therapeutic target for age-related metabolic disease, Crit. Rev. Biochem. Mol. Biol, vol.48, pp.397-408, 2013.

E. Pirinen, C. Canto, Y. S. Jo, L. Morato, H. Zhang et al., Pharmacological Inhibition of poly(ADP-ribose) polymerases improves fitness and mitochondrial function in skeletal muscle, Cell Metab, vol.19, pp.1034-1041, 2014.

R. H. Houtkooper, C. Canto, R. J. Wanders, and J. Auwerx, The secret life of NAD þ: an old metabolite controlling new metabolic signaling pathways, Endocr. Rev, vol.31, pp.194-223, 2010.

Y. Chen, L. L. Fu, X. Wen, X. Y. Wang, J. Liu et al., Sirtuin-3 (SIRT3), a therapeutic target with oncogenic and tumor-suppressive function in cancer, Cell Death Dis, vol.5, p.1047, 2014.

Y. T. Wu, S. B. Wu, and Y. H. Wei, Roles of sirtuins in the regulation of antioxidant defense and bioenergetic function of mitochondria under oxidative stress, Free Radic. Res, vol.48, pp.1070-1084, 2014.

X. Kong, R. Wang, Y. Xue, X. Liu, H. Zhang et al., Sirtuin 3, a new target of PGC-1alpha, plays an important role in the suppression of ROS and mitochondrial biogenesis, PloS One, vol.5, p.11707, 2010.

R. K. Chaturvedi and M. F. Beal, Mitochondria targeted therapeutic approaches in Parkinson's and Huntington's diseases, Mol. Cell. Neurosci, vol.55, pp.101-114, 2013.

G. N. Breningstall, J. Shoffner, and R. J. Patterson, Siblings with leukoencephalopathy, Semin. Pediatr. Neurol, vol.15, pp.212-215, 2008.

H. Pagniez-mammeri, A. Lombes, M. Brivet, H. Ogier-de-baulny, P. Landrieu et al., Rapid screening for nuclear genes mutations in isolated respiratory chain complex I defects, Mol. Genet. Metab, vol.96, pp.196-200, 2009.

H. A. Tuppen, V. E. Hogan, L. He, E. L. Blakely, L. Worgan et al., The p.M292T NDUFS2 mutation causes complex I-deficient Leigh syndrome in multiple families, Brain: J. Neurol, vol.133, pp.2952-2963, 2010.

Z. Assouline, M. Jambou, M. Rio, C. Bole-feysot, P. De-lonlay et al., A constant and similar assembly defect of mitochondrial respiratory chain complex I allows rapid identification of NDUFS4 mutations in patients with Leigh syndrome, Biochim. BIophys. Acta, vol.1822, pp.1062-1069, 2012.

S. Lebon, L. Minai, D. Chretien, J. Corcos, V. Serre et al., A novel mutation of the NDUFS7 gene leads to activation of a cryptic exon and impaired assembly of mitochondrial complex I in a patient with Leigh syndrome, Mol. Genet. Metab, vol.92, pp.104-108, 2007.

S. Lebon, D. Rodriguez, D. Bridoux, A. Zerrad, A. Rotig et al., A novel mutation in the human complex I NDUFS7 subunit associated with Leigh syndrome, Mol. Genet. Metab, vol.90, pp.379-382, 2007.