P. Rinaldo, D. Matern, and M. J. Bennett, Fatty acid oxidation disorders, Annu. Rev. Physiol, vol.64, pp.477-502, 2002.

B. S. Andresen, S. Olpin, B. J. Poorthuis, H. R. Scholte, C. Vianey-saban et al., Clear correlation of genotype with disease phenotype in very-long-chain acyl-CoA dehydrogenase deficiency, Am. J. Hum. Genet, vol.64, pp.479-494, 1999.

N. Gregersen, B. S. Andresen, M. J. Corydon, T. J. Corydon, R. K. Olsen et al., 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.

N. Gregersen, P. Bross, and B. S. Andresen, Genetic defects in fatty acid beta-oxidation and acyl-CoA dehydrogenases. Molecular pathogenesis and genotype-phenotype relationships, Eur. J. Biochem, vol.271, pp.470-482, 2004.

C. Vianey-saban, P. Divry, M. Brivet, M. Nada, M. T. Zabot et al., Mitochondrial very-long-chain acyl-coenzyme A dehydrogenase deficiency: clinical characteristics and diagnostic considerations in 30 patients, Clin. Chim. Acta, vol.269, pp.43-62, 1998.

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.

K. G. Sim, J. Hammond, and B. Wilcken, Strategies for the diagnosis of mitochondrial fatty acid beta-oxidation disorders, Clin. Chim. Acta, vol.323, pp.37-58, 2002.

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.

U. Spiekerkoetter, B. Sun, T. Zytkovicz, R. Wanders, A. W. Strauss et al., MS/MS-based newborn and family screening detects asymptomatic patients with very-long-chain acyl-CoA dehydrogenase deficiency, J Pediatr, vol.143, pp.335-342, 2003.

J. M. Saudubray, D. Martin, P. De-lonlay, G. Touati, F. Poggi-travert et al., Recognition and management of fatty acid oxidation defects: a series of 107 patients, J. Inherit. Metab. Dis, vol.22, pp.488-502, 1999.

C. R. Roe, L. Sweetman, D. S. Roe, F. David, and H. Brunengraber, Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride, J. Clin. Invest, vol.110, pp.259-269, 2002.

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.

J. Berger and D. E. Moller, The mechanisms of action of PPARs, Annu. Rev. Med, vol.53, pp.409-435, 2002.

J. Vamecq and N. Latruffe, Medical significance of peroxisome proliferator-activated receptors, Lancet, vol.354, pp.141-148, 1999.

R. M. Evans, G. D. Barish, and Y. X. Wang, PPARs and the complex journey to obesity, Nat. Med, vol.10, pp.355-361, 2004.

C. R. Roe and D. S. Roe, Recent developments in the investigations of inherited metabolic disorders using cultured human cells, Mol. Genet. Metab, vol.68, pp.243-257, 1999.

G. Sim, K. Carpenter, K. Hammond, J. Christodoulou, J. Wilcken et al., Quantitative fibroblast acylcarnitine profiles in mitochondrial fatty acid beta-oxidation defects: phenotype/metabolite correlations, Mol. Genet. Metab, vol.76, pp.327-334, 2002.

P. I. Eacho and P. S. Foxworthy, Inhibition of hepatic fatty acid oxidation by bezafibrate and bezafibroyl CoA, Biochem. Biophys. Res. Commun, vol.157, pp.1148-1153, 1988.

T. Aoyama, J. M. Peters, N. Iritani, T. Nakajima, K. Furihata et al., Altered constitutive expression of fatty acid-metabolizing enzymes in mice lacking the peroxisome proliferator-activated receptor alpha (PPARalpha), J. Biol. Chem, vol.273, pp.5678-5684, 1998.

J. M. Peters, T. Aoyama, A. M. Burns, and F. J. Gonzalez, Bezafibrate is a dual ligand for PPARalpha and PPARbeta: studies using null mice, Biochim. Biophys. Acta, vol.1632, pp.80-89, 2003.

L. F. Zhang, J. H. Ding, B. Z. Yang, G. C. He, and C. Roe, Characterization of the bidirectional promoter region between the human genes encoding VLCAD and PSD-95, Genomics, vol.82, pp.660-668, 2003.

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

N. J. Manning, S. E. Olpin, R. J. Pollitt, and J. Webley, 10-3H]myristic acids for the detection of defects of fatty acid oxidation in intact cultured fibroblasts, J. Inherit. Metab. Dis, vol.9, pp.58-68, 1990.

R. J. Wanders, P. Vreken, M. E. Den-boer, F. A. Wijburg, A. H. Van-gennip et al., Disorders of mitochondrial fatty acyl-CoA beta-oxidation, J. Inherit. Metab. Dis, vol.22, pp.442-487, 1999.

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