The molecular basis of human complex I deficiency, IUBMB Life, vol.63, pp.669-677, 2011. ,
Mitochondrial complex I deficiency: from organelle dysfunction to clinical disease, Brain: J. Neurol, vol.132, pp.833-842, 2009. ,
Complex I deficiencies in neurological disorders, Trends Mol. Med, vol.19, pp.61-69, 2013. ,
Molecular diagnosis in mitochondrial complex I deficiency using exome sequencing, J. Med. Genet, vol.49, pp.277-283, 2012. ,
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
Pharmacological targeting of mitochondrial complex I deficiency: the cellular level and beyond, Mitochondrion, vol.12, pp.57-65, 2012. ,
Complex I disorders: causes, mechanisms, and development of treatment strategies at the cellular level, Dev. Disabil. Res. Rev, vol.16, pp.175-182, 2010. ,
Resveratrol and clinical trials: the crossroad from in vitro studies to human evidence, Curr. Pharm. Des, vol.19, pp.6064-6093, 2013. ,
The molecular targets of resveratrol, Biochim. Biophys. Acta, vol.1852, pp.1114-1123, 2015. ,
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
Neuroprotective action of resveratrol, Biochim. Biophys. Acta, vol.1852, pp.1195-1201, 2015. ,
SIRT3, a pivotal actor in mitochondrial functions: metabolism, cell death and aging, Biochem. J, vol.444, pp.1-10, 2012. ,
Tumour suppressor SIRT3 deacetylates and activates manganese superoxide dismutase to scavenge ROS, EMBO Rep, vol.12, pp.534-541, 2011. ,
Calorie restriction reduces oxidative stress by SIRT3-mediated SOD2 activation, Cell Metab, vol.12, pp.662-667, 2010. ,
Sirt3-mediated deacetylation of evolutionarily conserved lysine 122 regulates MnSOD activity in response to stress, Mol. Cell, vol.40, pp.893-904, 2010. ,
Galactose enhances oxidative metabolism and reveals mitochondrial dysfunction in human primary muscle cells, PloS One, vol.6, p.28536, 2011. ,
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. ,
Spectrophotometric assay for complex I of the respiratory chain in tissue samples and cultured fibroblasts, Clin. Chem, vol.53, pp.729-734, 2007. ,
Determination of superoxide dismutase activity by purely chemical system based on NAD(P)H oxidation, Methods Enzymol, vol.186, pp.209-220, 1990. ,
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. ,
Mechanism for the protective effect of resveratrol against oxidative stress-induced neuronal death, Free Radic, Biol. Med, vol.49, pp.800-813, 2010. ,
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. ,
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. ,
Dietary resveratrol administration increases MnSOD expression and activity in mouse brain, Biochem. Biophys. Res. Commun, vol.372, pp.254-259, 2008. ,
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. ,
The dynamic regulation of NAD metabolism in mitochondria, Trends Endocrinol. Metab, vol.23, pp.420-428, 2012. ,
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
Pharmacological NAD-boosting strategies improve mitochondrial homeostasis in human complex I-Mutant fibroblasts, Mol. Pharmacol, vol.87, pp.965-971, 2015. ,
Effective treatment of mitochondrial myopathy by nicotinamide riboside, a vitamin B3, EBO Mol. Med, vol.6, pp.721-731, 2014. ,
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. ,
NAD(þ ) metabolism: a therapeutic target for age-related metabolic disease, Crit. Rev. Biochem. Mol. Biol, vol.48, pp.397-408, 2013. ,
Pharmacological Inhibition of poly(ADP-ribose) polymerases improves fitness and mitochondrial function in skeletal muscle, Cell Metab, vol.19, pp.1034-1041, 2014. ,
The secret life of NAD þ: an old metabolite controlling new metabolic signaling pathways, Endocr. Rev, vol.31, pp.194-223, 2010. ,
Sirtuin-3 (SIRT3), a therapeutic target with oncogenic and tumor-suppressive function in cancer, Cell Death Dis, vol.5, p.1047, 2014. ,
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. ,
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. ,
Mitochondria targeted therapeutic approaches in Parkinson's and Huntington's diseases, Mol. Cell. Neurosci, vol.55, pp.101-114, 2013. ,
Siblings with leukoencephalopathy, Semin. Pediatr. Neurol, vol.15, pp.212-215, 2008. ,
Rapid screening for nuclear genes mutations in isolated respiratory chain complex I defects, Mol. Genet. Metab, vol.96, pp.196-200, 2009. ,
The p.M292T NDUFS2 mutation causes complex I-deficient Leigh syndrome in multiple families, Brain: J. Neurol, vol.133, pp.2952-2963, 2010. ,
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. ,
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. ,
A novel mutation in the human complex I NDUFS7 subunit associated with Leigh syndrome, Mol. Genet. Metab, vol.90, pp.379-382, 2007. ,