E. P. Hoffman, R. H. Brown, and L. M. Kunkel, Dystrophin: the protein product of the Duchenne muscular dystrophy locus, Cell, vol.51, pp.919-928, 1987.

J. B. Bodensteiner and A. G. Engel, Intracellular calcium accumulation in Duchenne dystrophy and other myopathies A study of 567,000 muscle fibers in 114 biopsies, Neurology, vol.28, pp.439-439, 1978.

P. R. Turner, P. Fong, W. F. Denetclaw, and R. A. Steinhardt, Increased calcium influx in dystrophic muscle, The Journal of Cell Biology, vol.115, pp.1701-1712, 1991.

J. M. Alderton and R. A. Steinhardt, Calcium influx through calcium leak channels is responsible for the elevated levels of calciumdependent proteolysis in dystrophic myotubes, Journal of Biological Chemistry, vol.275, pp.9452-9460, 2000.

C. Vandebrouck, D. Martin, M. Colson-van-schoor, H. Debaix, and P. Gailly, Involvement of TRPC in the abnormal calcium influx observed in dystrophic (mdx) mouse skeletal muscle fibers, The Journal of cell biology, vol.158, pp.1089-1096, 2002.

M. J. Jackson, D. A. Jones, and R. H. Edwards, Measurements of calcium and other elements in muscle biopsy samples from patients with Duchenne muscular dystrophy, Clinica chimica acta, vol.147, pp.215-221, 1985.

I. A. Williams and D. G. Allen, Intracellular calcium handling in ventricular myocytes from mdx mice, American Journal of Physiology-Heart and Circulatory Physiology, vol.292, pp.846-855, 2007.

M. H. Disatnik, Evidence of oxidative stress in mdx mouse muscle: Studies of the pre-necrotic state, Journal of the Neurological Sciences, vol.161, pp.77-84, 1998.

J. W. Haycock, S. Mac-neil, P. Jones, J. B. Harris, and D. Mantle, Oxidative damage to muscle protein in Duchenne muscular dystrophy, Neuroreport, vol.8, pp.357-361, 1996.

R. W. Dudley, Sarcolemmal damage in dystrophin deficiency is modulated by synergistic interactions between mechanical and oxidative/nitrosative stresses, The American journal of pathology, vol.168, pp.1276-1287, 2006.

S. Messina, Lipid peroxidation inhibition blunts nuclear factor-?B activation, reduces skeletal muscle degeneration, and enhances muscle function in mdx mice, The American journal of pathology, vol.168, pp.918-926, 2006.

D. G. Allen, O. L. Gervasio, E. W. Yeung, and N. P. Whitehead, Calcium and the damage pathways in muscular dystrophy This article is one of a selection of papers published in this special issue on Calcium Signaling, Canadian journal of physiology and pharmacology, vol.88, pp.83-91, 2010.

M. Eagle, Survival in Duchenne muscular dystrophy: improvements in life expectancy since 1967 and the impact of home nocturnal ventilation, Neuromuscular Disorders, vol.12, pp.140-142, 2002.

C. A. Timpani, A. Hayes, and E. Rybalka, Revisiting the dystrophin-ATP connection: How half a century of research still implicates mitochondrial dysfunction in Duchenne Muscular Dystrophy aetiology, Medical Hypotheses, vol.85, pp.1021-1033, 2015.

A. Hayes and D. A. Williams, Beneficial effects of voluntary wheel running on the properties of dystrophic mouse muscle, Journal of applied physiology, vol.80, pp.670-679, 1996.

M. Onopiuk, Mutation in dystrophin-encoding gene affects energy metabolism in mouse myoblasts, Biochemical and Biophysical Research Communications, vol.386, pp.463-466, 2009.

E. Rybalka, C. A. Timpani, M. B. Cooke, A. D. Williams, and A. Hayes, Defects in Mitochondrial ATP Synthesis in Dystrophin-Deficient Mdx Skeletal Muscles May Be Caused by Complex I Insufficiency, PloS one, vol.9, p.115763, 2014.

C. M. Mcdonald, Idebenone reduces respiratory complications in patients with Duchenne muscular dystrophy, Neuromuscular Disorders, vol.26, pp.473-480, 2016.

E. Rybalka, C. A. Timpani, C. G. Stathis, A. Hayes, and M. B. Cooke, Metabogenic and nutriceutical approaches to address energy dysregulation and skeletal muscle wasting in duchenne muscular dystrophy, Nutrients, vol.7, pp.9734-9767, 2015.

C. Bonsett and A. Rudman, The dystrophin connection-ATP?, Medical Hypotheses, vol.38, pp.139-154, 1992.

P. Marshall, P. Williams, and G. Goldspink, Accumulation of collagen and altered fiber-type ratios as indicators of abnormal muscle gene expression in the mdx dystrophic mouse, Muscle & nerve, vol.12, pp.528-537, 1989.

H. Akima, Relationships of thigh muscle contractile and non-contractile tissue with function, strength, and age in boys with Duchenne muscular dystrophy, Neuromuscular Disorders, vol.22, pp.16-25, 2012.

H. K. Kim, T2 Mapping in Duchenne Muscular Dystrophy: Distribution of Disease Activity and Correlation with Clinical Assessments 1, Radiology, vol.255, pp.899-908, 2010.

J. R. Gooding, Adenylosuccinate Is an Insulin Secretagogue Derived from Glucose-Induced Purine Metabolism, Cell reports, vol.13, pp.157-167, 2015.

M. J. Glesby, E. Rosenmann, E. G. Nylen, and K. Wrogemann, Serum CK, calcium, magnesium, and oxidative phosphorylation in mdx mouse muscular dystrophy, Muscle & Nerve, vol.11, pp.852-856, 1988.

A. V. Kuznetsov, Impaired mitochondrial oxidative phosphorylation in skeletal muscle of the dystrophin-deficient mdx mouse, Molecular and cellular biochemistry, vol.183, pp.87-96, 1998.

M. Martens, L. Jankulovska, M. Neymark, and C. Lee, Impaired substrate utilization in mitochondria from strain 129 dystrophic mice, Biochimica et Biophysica Acta (BBA)-Bioenergetics, vol.589, pp.190-200, 1980.

S. K. Bhattacharya, P. L. Johnson, and J. H. Thakar, Reversal of impaired oxidative phosphorylation and calcium overloading in the in vitro cardiac mitochondria of CHF-146 dystrophic hamsters with hereditary muscular dystrophy, Journal of the Neurological Sciences, vol.120, pp.180-186, 1993.

V. Faist, J. König, H. Höger, and I. Elmadfa, Decreased mitochondrial oxygen consumption and antioxidant enzyme activities in skeletal muscle of dystrophic mice after low-intensity exercise, Annals of nutrition and metabolism, vol.45, pp.58-66, 2001.

E. Olson, P. Vignos, J. Woodlock, and T. Perry, Oxidative phosphorylation of skeletal muscle in human muscular dystrophy, J. Lab. Clin. Med, vol.71, p.23, 1968.

J. Griffin, Metabolic Profiling of Genetic Disorders: A Multitissue 1 H Nuclear Magnetic Resonance Spectroscopic and Pattern Recognition Study into Dystrophic Tissue, Analytical Biochemistry, vol.293, pp.16-21, 2001.

W. Chen, W. Huang, C. Chiu, Y. Chang, and C. Huang, Whey protein improves exercise performance and biochemical profiles in trained mice, Medicine and science in sports and exercise, vol.46, p.1517, 2014.

T. Shavlakadze, J. White, J. F. Hoh, N. Rosenthal, and M. D. Grounds, Targeted expression of insulin-like growth factor-I reduces early myofiber necrosis in dystrophic mdx mice, Molecular Therapy, vol.10, pp.829-843, 2004.

D. C. Sheehan and B. B. Hrapchak, Theory and practice of histotechnology, 1980.

C. A. Timpani, Attempting to Compensate for Reduced Neuronal Nitric Oxide Synthase Protein with Nitrate Supplementation Cannot Overcome Metabolic Dysfunction but Rather Has Detrimental Effects in Dystrophin-Deficient mdx Muscle, Neurotherapeutics, pp.1-18, 2016.

J. C. Sorensen, BGP-15 protects against Oxaliplatin-induced skeletal myopathy and mitochondrial reactive oxygen species production in mice, Frontiers in Pharmacology, vol.8, 2017.

C. A. Timpani, Attempting to compensate for reduced neuronal nitric oxide synthase protein with nitrate supplementation cannot overcome metabolic dysfunction but rather Has detrimental effects in dystrophin-deficient mdx muscle, Neurotherapeutics, vol.14, pp.429-446, 2017.

S. V. Brooks and J. A. Faulkner, Contractile properties of skeletal muscles from young, adult and aged mice, The Journal of physiology, vol.404, pp.71-82, 1988.

R. A. Schuh, K. C. Jackson, R. J. Khairallah, C. W. Ward, and E. E. Spangenburg, Measuring mitochondrial respiration in intact single muscle fibers, American Journal of Physiology -Regulatory, Integrative and Comparative Physiology, vol.302, pp.712-719, 2012.

C. Welinder and L. Ekblad, Coomassie Staining as Loading Control in Western Blot Analysis, Journal of Proteome Research, vol.10, pp.1416-1419, 2011.

A. Vigelsø-hansen, N. B. Andersen, and F. Dela, The relationship between skeletal muscle mitochondrial citrate synthase activity and whole body oxygen uptake adaptations in response to exercise training, International journal of physiology, vol.6, pp.84-101, 2014.

P. Srere,

, Methods in enzymology, vol.13, pp.3-11, 1969.

O. Lowry and J. Passonneau, , 1972.

C. G. Stathis, M. F. Carey, A. Hayes, A. P. Garnham, and R. J. Snow, Sprint training reduces urinary purine loss following intense exercise in humans, vol.31, pp.702-708, 2006.

C. Bonsett, A. Rudman, and A. Y. Elliott, Intracellular lipid in pseudohypertrophic muscular dystrophy tissue culture, J. Indiana State Med. Assoc, vol.72, pp.184-187, 1979.

H. Reichmann, H. Hoppeler, O. Mathieu-costello, F. Von-bergen, and D. Pette, Biochemical and ultrastructural changes of skeletal muscle mitochondria after chronic electrical stimulation in rabbits, Pflügers Archiv, vol.404, pp.1-9, 1985.

S. Larsen, Biomarkers of mitochondrial content in skeletal muscle of healthy young human subjects, The Journal of physiology, vol.590, pp.3349-3360, 2012.

J. M. Suárez-rivero, Mitochondrial Dynamics in Mitochondrial Diseases, Diseases, vol.5, p.1, 2016.

R. Armstrong and R. Phelps, Muscle fiber type composition of the rat hindlimb, American Journal of Anatomy, vol.171, pp.259-272, 1984.

C. A. Pickett-gies, R. C. Carlsen, L. J. Anderson, K. L. Angelos, and D. A. Walsh, Characterization of the isolated rat flexor digitorum brevis for the study of skeletal muscle phosphorylase kinase phosphorylation, Journal of Biological Chemistry, vol.262, pp.3227-3238, 1987.

T. J. Burkholder, B. Fingado, S. Baron, and R. L. Lieber, Relationship between muscle fiber types and sizes and muscle architectural properties in the mouse hindlimb, Journal of Morphology, vol.221, pp.177-190, 1994.

A. V. Kuznetsov, Striking Differences Between the Kinetics of Regulation of Respiration by ADP in Slow-Twitch and Fast-Twitch Muscles In Vivo, European Journal of Biochemistry, vol.241, pp.909-915, 1996.
URL : https://hal.archives-ouvertes.fr/inserm-00391362

K. Sahlin and S. Broberg, Adenine nucleotide depletion in human muscle during exercise: causality and significance of AMP deamination, International journal of sports medicine, vol.11, pp.62-67, 1990.

M. M. Mihaylova and R. J. Shaw, The AMP-activated protein kinase (AMPK) signaling pathway coordinates cell growth, autophagy, & metabolism, Nature cell biology, vol.13, p.1016, 2011.

K. Matsumura, J. M. Ervasti, K. Ohlendieck, S. D. Kahl, and K. P. Campbell, Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle, Nature, vol.360, pp.588-591, 1992.

V. Ljubicic, Chronic AMPK activation evokes the slow, oxidative myogenic program and triggers beneficial adaptations in mdx mouse skeletal muscle, Human molecular genetics, p.265, 2011.

G. M. Buyse, Efficacy of idebenone on respiratory function in patients with Duchenne muscular dystrophy not using glucocorticoids (DELOS): a double-blind randomised placebo-controlled phase 3 trial, The Lancet, vol.385, pp.1748-1757, 2015.

F. Mastaglia and B. Kakulas, Regeneration in Duchenne muscular dystrophy: a histological and histochemical study, Brain, vol.92, pp.809-818, 1969.

C. Pastoret and A. Sebille, Mdx mice show progressive weakness and muscle deterioration with age, Journal of the neurological sciences, vol.129, pp.97-105, 1995.

I. Arpan, T2 mapping provides multiple approaches for the characterization of muscle involvement in neuromuscular diseases: a cross-sectional study of lower leg muscles in 5-15-year-old boys with Duchenne muscular dystrophy, NMR in biomedicine, vol.26, pp.320-328, 2013.

T. A. Wren, S. Bluml, L. Tseng-ong, and V. Gilsanz, Three-point technique of fat quantification of muscle tissue as a marker of disease progression in Duchenne muscular dystrophy: preliminary study, American Journal of Roentgenology, vol.190, pp.8-12, 2008.

D. Cros, P. Harnden, J. Pellissier, and G. Serratrice, Muscle hypertrophy in Duchenne muscular dystrophy, Journal of neurology, vol.236, pp.43-47, 1989.

R. Willcocks, Longitudinal measurements of MRI-T 2 in boys with Duchenne muscular dystrophy: Effects of age and disease progression, Neuromuscular Disorders, vol.24, pp.393-401, 2014.

R. S. Vohra, Magnetic resonance assessment of hypertrophic and pseudo-hypertrophic changes in lower leg muscles of boys with Duchenne muscular dystrophy and their relationship to functional measurements, PloS one, vol.10, p.128915, 2015.

G. R. Coulton, J. E. Morgan, T. A. Partridge, and J. C. Sloper, The mdx mouse skeletal muscle myopathy: I. A histological, morphometric and biochemical investigation, Neuropathology and Applied Neurobiology, vol.14, pp.53-70, 1988.

C. Bonsett and A. Rudman, Duchenne's muscular dystrophy: a tissue culture perspective. Indiana medicine: the journal of the Indiana State, Medical Association, vol.77, p.446, 1984.

S. Rayavarapu, Identification of disease specific pathways using in vivo SILAC proteomics in dystrophin deficient mdx mouse, Molecular & cellular proteomics: MCP, vol.12, pp.1061-1073, 2013.

J. Dreyfus, G. Schapira, and F. Schapira, Biochemical study of muscle in progressive muscular dystrophy, J. Clin. Invest, vol.33, pp.794-797, 1954.

D. Mauro, S. Angelini, C. Catani, and C. , Enzymes of the glycogen cycle and glycolysis in various human neuromuscular disorders, J. Neurol. Neurosurg. Psychiat, vol.30, pp.411-415, 1967.

J. Hess, Phosphorylase activity and glycogen, glucose-6-phosphate, and lactic acid content of human skeletal muscle in various myopathies, J. Lab. Clin. Med, vol.66, pp.452-463, 1965.

M. M. Chi, Effect of Duchenne muscular dystrophy on enzymes of energy metabolism in individual muscle fibers, Metabolism, vol.36, pp.90113-90115, 1987.

D. I. Stapleton, Dysfunctional Muscle and Liver Glycogen Metabolism in mdx Dystrophic Mice, PLoS One, vol.9, 2014.

E. Ronzoni, L. Berg, and W. Landau, Enzyme studies in progressive muscular dystrophy, Res. Publ. Ass. nerv. ment. Dis, vol.38, pp.721-729, 1960.

D. Ellis, Intermediary metabolism of muscle in Duchenne muscular dystrophy, British Medical Bulletin, vol.36, pp.165-172, 1980.

J. K. Petell, N. A. Marshall, and H. G. Lebherz, Content and synthesis of several abundant glycolytic enzymes in skeletal muscles of normal and dystrophic mice, International Journal of Biochemistry, vol.16, pp.61-67, 1984.

Y. W. Chen, P. Zhao, R. Borup, and E. P. Hoffman, Expression profiling in the muscular dystrophies: identification of novel aspects of molecular pathophysiology, J. Cell. Biol, vol.151, pp.1321-1336, 2000.

S. Carberry, H. Brinkmeier, Y. Zhang, C. K. Winkler, and K. Ohlendieck, Comparative proteomic profiling of soleus, extensor digitorum longus, flexor digitorum brevis and interosseus muscles from the mdx mouse model of Duchenne muscular dystrophy, International Journal of Molecular Medicine, 2013.

V. Ion??escu, N. Luca, and O. Vuia, Respiratory control and oxidative phosphorylation in the dystrophic muscle, Acta Neurologica Scandinavica, vol.43, pp.564-572, 1967.

E. G. Nylen and K. Wrogemann, Mitochondrial calcium content and oxidative phosphorylation in heart and skeletal muscle of dystrophic mice, Experimental neurology, vol.80, pp.69-80, 1983.

A. Chinet, P. Even, and A. Decrouy, Dystrophin-dependent efficiency of metabolic pathways in mouse skeletal muscles, Cellular and Molecular Life Sciences, vol.50, pp.602-605, 1994.

A. Cao, A. Macciotta, G. Fiorelli, P. Mannucci, and G. Idéo, Chromatographic and electrophoretic pattern of lactate and malate dehydrogenase in normal human adult and foetal muscle and in muscle of patients affected by Duchenne muscular dystrophy, Enzymologia biologica et clinica, vol.7, pp.156-166, 1965.

T. Akiba, K. Hiragi, and S. Tuboi, Intracellular distribution of fumarase in various animals, Journal of biochemistry, vol.96, pp.189-195, 1984.

S. Lee, Mitochondrial fission and fusion mediators, hFis1 and OPA1, modulate cellular senescence, Journal of Biological Chemistry, vol.282, pp.22977-22983, 2007.

P. H. Willems, R. Rossignol, C. E. Dieteren, M. P. Murphy, and W. J. Koopman, Redox homeostasis and mitochondrial dynamics, Cell metabolism, vol.22, pp.207-218, 2015.

R. J. Khairallah, Microtubules underlie dysfunction in duchenne muscular dystrophy, Science signaling, vol.5, 2012.

L. Austin, Potential oxyradical damage and energy status in individual muscle fibres from degenerating muscle diseases, Neuromuscular Disorders, vol.2, pp.27-33, 1992.

R. Pinto and W. Bartley, The effect of age and sex on glutathione reductase and glutathione peroxidase activities and on aerobic glutathione oxidation in rat liver homogenates, Biochemical Journal, vol.112, pp.109-115, 1969.

A. Laplante, G. Vincent, M. Poirier, and C. Des-rosiers, Effects and metabolism of fumarate in the perfused rat heart. A 13C mass isotopomer study, American Journal of Physiology-Endocrinology And Metabolism, vol.272, pp.74-82, 1997.

N. Raimundo, B. E. Baysal, and G. S. Shadel, Revisiting the TCA cycle: signaling to tumor formation, Trends in molecular medicine, vol.17, pp.641-649, 2011.

U. Sharma, S. Atri, M. Sharma, C. Sarkar, and N. Jagannathan, Skeletal muscle metabolism in Duchenne muscular dystrophy (DMD): an in-vitro proton NMR spectroscopy study, Magnetic resonance imaging, vol.21, pp.145-153, 2003.

C. H. Lin, Fatty acid oxidation by skeletal muscle mitochondria in Duchenne muscular dystrophy, Life Sci. II, vol.11, pp.355-362, 1972.

J. B. Shumate, J. E. Carroll, M. H. Brooke, and R. M. Choksi, Palmitate oxidation in human muscle: comparison to CPT and carnitine, Muscle & nerve, vol.5, pp.226-231, 1982.

J. E. Carroll, B. J. Norris, M. H. Brooke, and . Defective, U-14 C] palmitic acid oxidation in Duchenne muscular dystrophy, Neurology, vol.35, pp.96-97, 1985.

C. Kang, C. A. Goodman, T. A. Hornberger, and L. L. Ji, PGC-1? overexpression by in vivo transfection attenuates mitochondrial deterioration of skeletal muscle caused by immobilization, The FASEB Journal, vol.29, pp.4092-4106, 2015.