A. Berardo, S. Dimauro, and M. Hirano, A diagnostic algorithm for metabolic myopathies, Curr. Neurol. Neurosci. Rep, vol.10, pp.118-126, 2010.

E. C. Smith, A. El-gharbawy, and D. D. Koeberl, Metabolic myopathies: clinical features and diagnostic approach, Rheum. Dis. Clin. North Am, vol.37, pp.201-217, 2011.

J. P. Bonnefont, F. Djouadi, C. Prip-buus, S. Gobin, A. Munnich et al., Carnitine palmitoyltransferases 1 and 2: biochemical, molecular and medical aspects, Mol. Aspects. Med, vol.25, pp.495-520, 2004.

S. Schiaffino and C. Reggiani, Fiber types in mammalian skeletal muscles, Physiol. Rev, vol.91, pp.1447-1531, 2011.

B. Egan and J. R. Zierath, Exercise metabolism and the molecular regulation of skeletal muscle adaptation, Cell. Metab, vol.17, pp.162-184, 2013.

H. Hoppeler, Molecular networks in skeletal muscle plasticity, J. Exp. Biol, vol.219, pp.205-213, 2016.

B. Kupr and C. Handschin, Complex coordination of cell plasticity by a PGC-1alpha-controlled transcriptional network in skeletal muscle, Front. Physiol, vol.6, p.325, 2015.

R. Mounier, M. Theret, L. Lantier, M. Foretz, and B. Viollet, Expanding roles for AMPK in skeletal muscle plasticity, Trends Endocrinol. Metab, vol.26, pp.275-286, 2015.
URL : https://hal.archives-ouvertes.fr/inserm-01171734

A. M. Sanchez, R. B. Candau, A. Csibi, A. F. Pagano, A. Raibon et al., The role of AMP-activated protein kinase in the coordination of skeletal muscle turnover and energy homeostasis, Am. J. Physiol. Cell. Physiol, vol.303, pp.475-485, 2012.
URL : https://hal.archives-ouvertes.fr/hal-02652061

C. Handschin and B. M. Spiegelman, Peroxisome proliferator-activated receptor gamma coactivator 1 coactivators, energy homeostasis, and metabolism, Endocr. Rev, vol.27, pp.728-735, 2006.

R. C. Scarpulla, R. B. Vega, and D. P. Kelly, Transcriptional integration of mitochondrial biogenesis, Trends Endocrinol. Metab, vol.23, pp.459-466, 2012.

Z. Wu, P. Puigserver, U. Andersson, C. Zhang, G. Adelmant et al., Mechanisms controlling mitochondrial biogenesis and respiration through the thermogenic coactivator PGC-1, Cell, vol.98, pp.115-124, 1999.

J. Lin, H. Wu, P. T. Tarr, C. Y. Zhang, Z. Wu et al., Transcriptional co-activator PGC-1 alpha drives the formation of slow-twitch muscle fibres, Nature, vol.418, pp.797-801, 2002.

P. J. Fernandez-marcos and J. Auwerx, Regulation of PGC-1alpha, a nodal regulator of mitochondrial biogenesis, Am. J. Clin. Nutr, vol.93, pp.884-890, 2011.

C. Handschin, J. Rhee, J. Lin, P. T. Tarr, and B. M. Spiegelman, An autoregulatory loop controls peroxisome proliferator-activatedreceptorgammacoactivator1alpha expression in muscle, Proc. Natl. Acad. Sci. U S A, vol.100, pp.7111-7116, 2003.

M. Schuler, F. Ali, C. Chambon, D. Duteil, J. M. Bornert et al., PGC1alpha expression is controlled in skeletal muscles by PPARbeta, whose ablation results in fiber-type switching, obesity, and type 2 diabetes, Cell. Metab, vol.4, pp.407-414, 2006.
URL : https://hal.archives-ouvertes.fr/hal-00188136

D. M. Thomson, S. T. Herway, N. Fillmore, H. Kim, J. D. Brown et al., AMP-activated protein kinase phosphorylates transcription factors of the CREB family, J. Appl. Physiol, vol.104, pp.429-438, 1985.

S. Jager, C. Handschin, J. St-pierre, and B. M. Spiegelman, AMP-activated protein kinase (AMPK) action in skeletal muscle via direct phosphorylation of PGC-1alpha, Proc. Natl. Acad. Sci. U S A, vol.104, pp.12017-12022, 2007.

C. Canto and J. Auwerx, AMP-activated protein kinase and its downstream transcriptional pathways, Cell. Mol. Life Sci, vol.67, pp.3407-3423, 2010.

S. Herzig and R. J. Shaw, AMPK: guardian of metabolism and mitochondrial homeostasis, Nat. Rev. Mol. Cell. Biol, vol.19, pp.121-135, 2018.

E. Q. Toyama, S. Herzig, J. Courchet, T. L. Lewis, O. C. Loson et al., Metabolism. AMP-activated protein kinase mediates mitochondrial fission in response to energy stress, Science, vol.351, pp.275-281, 2016.

A. M. Fritzen, A. B. Madsen, M. Kleinert, J. T. Treebak, A. M. Lundsgaard et al., Regulation of autophagy in human skeletal muscle: effects of exercise, exercise training and insulin stimulation, J. Physiol, vol.594, pp.745-761, 2016.

J. S. Ju, S. I. Jeon, J. Y. Park, J. Y. Lee, S. C. Lee et al., Autophagy plays a role in skeletal muscle mitochondrial biogenesis in an endurance exercise-trained condition, J. Physiol. Sci, vol.66, pp.417-430, 2016.

Y. Kim and D. A. Hood, Regulation of the autophagy system during chronic contractile activity-induced muscle adaptations, Physiol. Rep, vol.5, 2017.

V. A. Lira, M. Okutsu, M. Zhang, N. P. Greene, R. C. Laker et al., Autophagy is required for exercise training-induced skeletal muscle adaptation and improvement of physical performance, FASEB J, vol.27, pp.4184-4193, 2013.

A. C. Daugan, .. Lamotte, Y. Mirguet, and O. , Patent WO 2012119978A1 Quinolinone derivatives as activators of AMPK, 2012.

M. J. Sanders, P. O. Grondin, B. D. Hegarty, M. A. Snowden, and D. Carling, Investigating the mechanism for AMP activation of the AMP-activated protein kinase cascade, Biochem. J, vol.403, pp.139-148, 2007.
URL : https://hal.archives-ouvertes.fr/hal-00478681

K. H. Carpenter and V. Wiley, Application of tandem mass spectrometry to biochemical genetics and newborn screening, Clin. Chim. Acta, vol.322, pp.1-10, 2002.

J. J. Shen, D. Matern, D. S. Millington, S. Hillman, M. D. Feezor et al., Acylcarnitines in fibroblasts of patients with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and other fatty acid oxidation disorders, J. Inherit. Metab. Dis, vol.23, pp.27-44, 2000.

T. E. Jensen, R. Leutert, S. T. Rasmussen, J. R. Mouatt, M. L. Christiansen et al., EMGnormalised kinase activation during exercise is higher in human gastrocnemius compared to soleus muscle, PLoS One, vol.7, p.31054, 2012.

J. N. Nielsen, K. J. Mustard, D. A. Graham, H. Yu, C. S. Macdonald et al., -AMP-activated protein kinase activity and subunit expression in exercisetrained human skeletal muscle, J. Appl. Physiol, vol.5, pp.631-641, 1985.

V. Martinez-redondo, A. T. Pettersson, and J. L. Ruas, The hitchhiker's guide to PGC-1alpha isoform structure and biological functions, Diabetologia, vol.58, pp.1969-1977, 2015.

K. Baar, A. R. Wende, T. E. Jones, M. Marison, L. A. Nolte et al., Adaptations of skeletal muscle to exercise: rapid increase in the transcriptional coactivator PGC-1, FASEB J, vol.16, pp.1879-1886, 2002.

M. Ydfors, H. Fischer, H. Mascher, E. Blomstrand, J. Norrbom et al., The truncated splice variants, NT-PGC-1alpha and PGC-1alpha4, increase with both endurance and resistance exercise in human skeletal muscle, Physiol. Rep, vol.1, p.140, 2013.

Y. Zhang, P. Huypens, A. W. Adamson, J. S. Chang, T. M. Henagan et al., Alternative mRNA splicing produces a novel biologically active short isoform of PGC-1alpha, 2009.

, J. Biol. Chem, vol.284, pp.32813-32826

R. K. Olsen, N. Cornelius, and N. Gregersen, Genetic and cellular modifiers of oxidative stress: what can we learn from fatty acid oxidation defects?, Mol. Genet. Metab, p.110, 2013.

S. K. Powers, J. Duarte, A. N. Kavazis, and E. E. Talbert, Reactive oxygen species are signalling molecules for skeletal muscle adaptation, Exp. Physiol, vol.95, pp.1-9, 2010.

D. Kang, S. H. Kim, and N. Hamasaki, Mitochondrial transcription factor A (TFAM): roles in maintenance of mtDNA and cellular functions, Mitochondrion, vol.7, pp.39-44, 2007.

E. Q. Toyama, S. Herzig, J. Courchet, T. L. Lewis, . Jr et al., Metabolism. AMP-activated protein kinase mediates mitochondrial fission in response to energy stress, Science, vol.351, pp.275-281, 2016.

J. Bastin, Regulation of mitochondrial fatty acid betaoxidation in human: what can we learn from inborn fatty acid beta-oxidation deficiencies?, Biochimie, vol.96, pp.113-120, 2014.

J. P. Bonnefont, J. Bastin, A. Behin, and F. Djouadi, Bezafibrate for treatment of an inborn mitochondrial ßoxidation defect, N. Engl. J. Med, vol.360, pp.838-840, 2009.

J. P. Bonnefont, J. Bastin, P. Laforet, F. Aubey, A. Mogenet et al., Long-term follow-up of bezafibrate treatment in patients with the myopathic form of carnitine palmitoyltransferase 2 deficiency, Clin. Pharmacol. Ther, vol.88, pp.101-108, 2010.

J. Bastin, A. Lopes-costa, and F. Djouadi, Exposure to resveratrol triggers pharmacological correction of fatty acid utilization in human fatty acid oxidation-deficient fibroblasts, Hum. Mol. Genet, vol.20, pp.2048-2057, 2011.

F. Djouadi, F. Aubey, D. Schlemmer, and J. Bastin, Peroxisome proliferator activated receptor delta (PPAR?) agonist but not PPAR? corrects carnitine palmitoyl transferase 2 deficiency in human muscle cells, J. Clin. Endocrinol. Metab, vol.90, pp.1791-1797, 2005.

F. Djouadi, J. P. Bonnefont, L. Thuillier, V. Droin, N. Khadom et al., Correction of fatty acid oxidation in carnitine palmitoyl transferase 2-deficient cultured skin fibroblasts by bezafibrate, Pediatr. Res, vol.54, pp.446-451, 2003.

A. Lopes-costa, C. Le-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.

I. Dikic and Z. Elazar, Mechanism and medical implications of mammalian autophagy, Nat. Rev. Mol. Cell. Biol, vol.19, pp.349-364, 2018.

B. Guigas, N. Taleux, M. Foretz, D. Detaille, F. Andreelli et al., AMP-activated protein kinaseindependent inhibition of hepatic mitochondrial oxidative phosphorylation by AICA riboside, Biochem. J, vol.404, pp.499-507, 2007.
URL : https://hal.archives-ouvertes.fr/hal-00478741

E. Barbieri and P. Sestili, Reactive oxygen species in skeletal muscle signaling, J. Signal Transduct, vol.982794, pp.1-17, 2012.

L. L. Ji, C. Kang, and Y. Zhang, Exercise-induced hormesis and skeletal muscle health. Free Radic, Biol. Med, vol.98, pp.113-122, 2016.

M. Kozakowska, K. Pietraszek-gremplewicz, A. Jozkowicz, and J. Dulak, The role of oxidative stress in skeletal muscle injury and regeneration: focus on antioxidant enzymes, J. Muscle Res. Cell. Motil, vol.36, pp.377-393, 2015.

M. Moulin and A. Ferreiro, Muscle redox disturbances and oxidative stress as pathomechanisms and therapeutic targets in early-onset myopathies, Semin. Cell. Dev. Biol, vol.64, pp.213-223, 2017.

F. Djouadi, J. P. Bonnefont, A. Munnich, and J. Bastin, Characterization of fatty acid oxidation in human muscle mitochondria and myoblasts, Mol. Genet. Metab, vol.78, pp.112-118, 2003.

O. H. Lowry, N. J. Rosebrough, A. L. Farr, and R. J. Randall, Protein measurement with the folin phenol reagent, J. Biol. Chem, vol.193, pp.265-275, 1951.

D. Carling, P. R. Clarke, V. A. Zammit, and D. G. Hardie, Purification and characterization of the AMP-activated protein kinase. Copurification of acetyl-CoA carboxylase kinase and 3-hydroxy-3-methylglutaryl-CoA reductase kinase activities, Eur. J. Biochem, vol.186, pp.129-136, 1989.

S. P. Davies, D. Carling, and D. G. Hardie, Tissue distribution of the AMP-activated protein kinase, and lack of activation by cyclic-AMP-dependent protein kinase, studied using a specific and sensitive peptide assay, Eur. J. Biochem, vol.186, pp.123-128, 1989.