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Should the beneficial impact of bezafibrate on fatty acid oxidation disorders be questioned?
Bastin J, Bonnefont JP, Djouadi F, Bresson JL. Bastin J, et al. J Inherit Metab Dis. 2015 Mar;38(2):371-2. doi: 10.1007/s10545-014-9775-7. Epub 2014 Oct 14. J Inherit Metab Dis. 2015. PMID: 25310995 No abstract available.
A gender-related defect in lipid metabolism and glucose homeostasis in peroxisome proliferator- activated receptor alpha- deficient mice.
Djouadi F, Weinheimer CJ, Saffitz JE, Pitchford C, Bastin J, Gonzalez FJ, Kelly DP. Djouadi F, et al. J Clin Invest. 1998 Sep 15;102(6):1083-91. doi: 10.1172/JCI3949. J Clin Invest. 1998. PMID: 9739042 Free PMC article.
[Mitochondrial beta-oxidation of fatty acids: an essential metabolic pathway of muscular function].
de Lonlay P, Djouadi F, Bonnefont JP, Saudubray JM, Bastin J. de Lonlay P, et al. Arch Pediatr. 2002 May;9 Suppl 2:175s-178s. doi: 10.1016/s0929-693x(01)00881-8. Arch Pediatr. 2002. PMID: 12108259 Review. French. No abstract available.
Characterization of fatty acid oxidation in human muscle mitochondria and myoblasts.
Djouadi F, Bonnefont JP, Munnich A, Bastin J. Djouadi F, et al. Mol Genet Metab. 2003 Feb;78(2):112-8. doi: 10.1016/s1096-7192(03)00017-9. Mol Genet Metab. 2003. PMID: 12618083
Correction of fatty acid oxidation in carnitine palmitoyl transferase 2-deficient cultured skin fibroblasts by bezafibrate.
Djouadi F, Bonnefont JP, Thuillier L, Droin V, Khadom N, Munnich A, Bastin J. Djouadi F, et al. Pediatr Res. 2003 Oct;54(4):446-51. doi: 10.1203/01.PDR.0000083001.91588.BB. Epub 2003 Jul 2. Pediatr Res. 2003. PMID: 12840153
Carnitine palmitoyltransferases 1 and 2: biochemical, molecular and medical aspects.
Bonnefont JP, Djouadi F, Prip-Buus C, Gobin S, Munnich A, Bastin J. Bonnefont JP, et al. Mol Aspects Med. 2004 Oct-Dec;25(5-6):495-520. doi: 10.1016/j.mam.2004.06.004. Mol Aspects Med. 2004. PMID: 15363638 Review.
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.
Djouadi F, Aubey F, Schlemmer D, Ruiter JP, Wanders RJ, Strauss AW, Bastin J. Djouadi F, et al. Hum Mol Genet. 2005 Sep 15;14(18):2695-703. doi: 10.1093/hmg/ddi303. Epub 2005 Aug 22. Hum Mol Genet. 2005. PMID: 16115821
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