Mitochondrial fatty acid oxidation defects-remaining challenges, J. Inherit. Metab. Dis, vol.31, pp.643-657, 2008. ,
Pathophysiology of fatty acid oxidation disorders, J. Inherit. Metab. Dis, 2009. ,
Carnitine palmitoyltransferase II deficiency: a clinical, biochemical, and molecular review, Lab. Invest, vol.83, pp.1543-1554, 2003. ,
Carnitine palmitoyltransferases 1 and 2: biochemical, molecular and medical aspects, Mol. Aspects Med, vol.25, pp.495-520, 2004. ,
Minireview: lipid metabolism, metabolic diseases, and peroxisome proliferator-activated receptors, Endocrinology, vol.144, pp.2201-2207, 2003. ,
Dual and pan-peroxisome proliferator-activated receptors (PPAR) co-agonism: the bezafibrate lessons, Cardiovasc. Diabetol, vol.4, p.14, 2005. ,
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
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. ,
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
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
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. ,
PPAR alpha-activation results in enhanced carnitine biosynthesis and OCTN2-mediated hepatic carnitine accumulation, Biochim. Biophys. Acta, vol.1767, pp.1134-1142, 2007. ,
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. ,
Regulation of muscle fiber type and running endurance by PPARdelta, PLoS Biol, vol.2, p.294, 2004. ,
PPARs: transcription factors controlling lipid and lipoprotein metabolism, Ann. N. Y. Acad. Sci, vol.967, pp.7-18, 2002. ,
Drug treatment of lipid disorders, N. Engl. J. Med, vol.341, pp.498-511, 1999. ,
Peroxisome proliferator-activated receptors (PPARS): regulators of gene expression in heart and skeletal muscle, Acta Physiol. Scand, vol.178, pp.425-434, 2003. ,
PPAR delta: a dagger in the heart of the metabolic syndrome, J. Clin. Invest, vol.116, pp.590-597, 2006. ,
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. ,
A mitochondrial tRNA aspartate mutation causing isolated mitochondrial myopathy, Am. J. Med. Genet. A, vol.137, pp.170-175, 2005. ,
Task force guidelines handbook: EFNS guidelines on diagnosis and management of fatty acid mitochondrial disorders, Eur. J. Neurol, vol.13, pp.923-929, 2006. ,
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
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
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. ,
Correlation between genotype, metabolic data, and clinical presentation in carnitine palmitoyltransferase 2 (CPT2) deficiency, Hum. Mutat, vol.21, pp.493-501, 2003. ,
Recognition and management of fatty acid oxidation defects: a series of 107 patients, J. Inherit. Metab. Dis, vol.22, pp.488-502, 1999. ,
Protein measurement with the Folin phenol reagent, J. Biol. Chem, vol.193, pp.265-275, 1951. ,
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
Rapid diagnosis of long chain and medium chain fatty acid oxidation disorders using lymphocytes, Ann. Clin. Biochem, vol.32, pp.154-159, 1995. ,
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. ,
Lipid-lowering drugs and risk of myopathy: a population-based follow-up study, Epidemiology, vol.12, pp.565-569, 2001. ,
Fenofibrate-induced hyperhomocysteinemia may be prevented by folate co-administration ,
, Eur. J. Clin. Pharmacol, vol.59, pp.367-371, 2003.
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. ,