N. Gregersen, B. S. Andresen, C. B. Pedersen, R. K. Olsen, T. J. Corydon et al., Mitochondrial fatty acid oxidation defects-remaining challenges, J. Inherit. Metab. Dis, vol.31, pp.643-657, 2008.

M. J. Bennett, Pathophysiology of fatty acid oxidation disorders, J. Inherit. Metab. Dis, 2009.

E. Sigauke, D. Rakheja, K. Kitson, and M. Bennett, Carnitine palmitoyltransferase II deficiency: a clinical, biochemical, and molecular review, Lab. Invest, vol.83, pp.1543-1554, 2003.

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.

C. H. Lee, P. Olson, and R. M. Evans, Minireview: lipid metabolism, metabolic diseases, and peroxisome proliferator-activated receptors, Endocrinology, vol.144, pp.2201-2207, 2003.

A. Tenenbaum, M. Motro, and E. Z. Fisman, Dual and pan-peroxisome proliferator-activated receptors (PPAR) co-agonism: the bezafibrate lessons, Cardiovasc. Diabetol, vol.4, p.14, 2005.

F. Djouadi, Correction of fatty acid oxidation in carnitine palmitoyl transferase 2-deficient cultured skin fibroblasts by bezafibrate, Pediatr. Res, vol.54, pp.446-451, 2003.
URL : https://hal.archives-ouvertes.fr/inserm-02896299

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

J. P. Bonnefont, J. Bastin, A. Behin, and F. Djouadi, Bezafibrate for an inborn mitochondrial beta-oxidation defect, N. Engl. J. Med, vol.360, pp.838-840, 2009.
URL : https://hal.archives-ouvertes.fr/inserm-02896232

F. Djouadi, .. 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.
URL : https://hal.archives-ouvertes.fr/inserm-02896304

T. V. Perneger, A. Leplège, .. F. Etter, and A. Rougemont, Validation of a Frenchlanguage version of the MOS 36-Item Short Form Health Survey (SF-36) in young healthy adults, J. Clin. Epidemiol, vol.48, pp.1051-1060, 1995.

N. Van-vlies, S. Ferdinandusse, M. Turkenburg, R. J. Wanders, and F. M. Vaz, PPAR alpha-activation results in enhanced carnitine biosynthesis and OCTN2-mediated hepatic carnitine accumulation, Biochim. Biophys. Acta, vol.1767, pp.1134-1142, 2007.

S. Luquet, Roles of peroxisome proliferator-activated receptor delta (PPARdelta) in the control of fatty acid catabolism. A new target for the treatment of metabolic syndrome, Biochimie, vol.86, pp.833-837, 2004.

Y. X. Wang, Regulation of muscle fiber type and running endurance by PPARdelta, PLoS Biol, vol.2, p.294, 2004.

V. Bocher, I. Pineda-torra, .. C. Fruchart, and B. Staels, PPARs: transcription factors controlling lipid and lipoprotein metabolism, Ann. N. Y. Acad. Sci, vol.967, pp.7-18, 2002.

R. H. Knopp, Drug treatment of lipid disorders, N. Engl. J. Med, vol.341, pp.498-511, 1999.

A. J. Gilde and M. Van-bilsen, Peroxisome proliferator-activated receptors (PPARS): regulators of gene expression in heart and skeletal muscle, Acta Physiol. Scand, vol.178, pp.425-434, 2003.

G. D. Barish, V. A. Narkar, and R. M. Evans, PPAR delta: a dagger in the heart of the metabolic syndrome, J. Clin. Invest, vol.116, pp.590-597, 2006.

N. Gregersen, Mutation analysis in mitochondrial fatty acid oxidation defects: Exemplified by acyl-CoA dehydrogenase deficiencies, with special focus on genotype-phenotype relationship, Hum. Mutat, vol.18, pp.169-189, 2001.

S. Seneca, A mitochondrial tRNA aspartate mutation causing isolated mitochondrial myopathy, Am. J. Med. Genet. A, vol.137, pp.170-175, 2005.

C. Angelini, A. Federico, H. Reichmann, A. Lombes, P. Chinnery et al., Task force guidelines handbook: EFNS guidelines on diagnosis and management of fatty acid mitochondrial disorders, Eur. J. Neurol, vol.13, pp.923-929, 2006.

S. Gobin-limballe, Genetic basis for correction of very-long-chain acyl-coenzyme A dehydrogenase deficiency by bezafibrate in patient fibroblasts: toward a genotype-based therapy, Am. J. Hum. Genet, vol.81, pp.1133-1143, 2007.
URL : https://hal.archives-ouvertes.fr/inserm-02896262

J. Bastin, F. Aubey, A. Rötig, A. Munnich, and F. Djouadi, Activation of peroxisome proliferator-activated receptor pathway stimulates the mitochondrial respiratory chain and can correct deficiencies in patients' cells lacking its components, J. Clin. Endocrinol. Metab, vol.93, pp.1433-1441, 2008.
URL : https://hal.archives-ouvertes.fr/inserm-02896255

U. Risérus, Activation of peroxisome proliferator-activated receptor (PPAR)delta promotes reversal of multiple metabolic abnormalities, reduces oxidative stress, and increases fatty acid oxidation in moderately obese men, Diabetes, vol.57, pp.332-339, 2008.

L. Thuillier, Correlation between genotype, metabolic data, and clinical presentation in carnitine palmitoyltransferase 2 (CPT2) deficiency, Hum. Mutat, vol.21, pp.493-501, 2003.

J. M. Saudubray, Recognition and management of fatty acid oxidation defects: a series of 107 patients, J. Inherit. Metab. Dis, vol.22, pp.488-502, 1999.

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.

F. Djouadi, Bezafibrate increases very-long-chain acyl-CoA dehydrogenase protein and mRNA expression in deficient fibroblasts and is a potential therapy for fatty acid oxidation disorders, Hum. Mol. Genet, vol.14, pp.2695-2703, 2005.
URL : https://hal.archives-ouvertes.fr/inserm-02896287

M. Brivet, A. Slama, .. M. Saudubray, A. Legrand, and A. Lemonnier, Rapid diagnosis of long chain and medium chain fatty acid oxidation disorders using lymphocytes, Ann. Clin. Biochem, vol.32, pp.154-159, 1995.

F. Demaugre, .. P. Bonnefont, M. Colonna, C. Cepanec, .. P. Leroux et al., Infantile form of carnitine palmitoyltransferase II deficiency with hepatomuscular symptoms and sudden death. Physiopathological approach to carnitine palmitoyltransferase II deficiencies, J. Clin. Invest, vol.87, pp.859-864, 1991.

D. Gaist, L. A. Rodríguez, C. Huerta, . Hallas, and S. H. Sindrup, Lipid-lowering drugs and risk of myopathy: a population-based follow-up study, Epidemiology, vol.12, pp.565-569, 2001.

O. Mayer, J. Simon, L. Holubec, R. Pikner, and I. Subrt, Fenofibrate-induced hyperhomocysteinemia may be prevented by folate co-administration

, Eur. J. Clin. Pharmacol, vol.59, pp.367-371, 2003.

J. Najib, Fenofibrate in the treatment of dyslipidemia: a review of the data as they relate to the new suprabioavailable tablet formulation, Clin. Ther, vol.24, pp.2022-2050, 2002.