140 related articles for article (PubMed ID: 12840153)
1. 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
Pediatr Res; 2003 Oct; 54(4):446-51. PubMed ID: 12840153
[TBL] [Abstract][Full Text] [Related]
2. Peroxisome proliferator activated receptor delta (PPARdelta) agonist but not PPARalpha corrects carnitine palmitoyl transferase 2 deficiency in human muscle cells.
Djouadi F; Aubey F; Schlemmer D; Bastin J
J Clin Endocrinol Metab; 2005 Mar; 90(3):1791-7. PubMed ID: 15613406
[TBL] [Abstract][Full Text] [Related]
3. 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
Hum Mol Genet; 2005 Sep; 14(18):2695-703. PubMed ID: 16115821
[TBL] [Abstract][Full Text] [Related]
4. Effect of heat stress and bezafibrate on mitochondrial beta-oxidation: comparison between cultured cells from normal and mitochondrial fatty acid oxidation disorder children using in vitro probe acylcarnitine profiling assay.
Li H; Fukuda S; Hasegawa Y; Kobayashi H; Purevsuren J; Mushimoto Y; Yamaguchi S
Brain Dev; 2010 May; 32(5):362-70. PubMed ID: 19589653
[TBL] [Abstract][Full Text] [Related]
5. Exposure to resveratrol triggers pharmacological correction of fatty acid utilization in human fatty acid oxidation-deficient fibroblasts.
Bastin J; Lopes-Costa A; Djouadi F
Hum Mol Genet; 2011 May; 20(10):2048-57. PubMed ID: 21378393
[TBL] [Abstract][Full Text] [Related]
6. Potential of fibrates in the treatment of fatty acid oxidation disorders: revival of classical drugs?
Djouadi F; Aubey F; Schlemmer D; Gobin S; Laforet P; Wanders RJ; Strauss AW; Bonnefont JP; Bastin J
J Inherit Metab Dis; 2006; 29(2-3):341-2. PubMed ID: 16763897
[TBL] [Abstract][Full Text] [Related]
7. Functional analysis of iPSC-derived myocytes from a patient with carnitine palmitoyltransferase II deficiency.
Yasuno T; Osafune K; Sakurai H; Asaka I; Tanaka A; Yamaguchi S; Yamada K; Hitomi H; Arai S; Kurose Y; Higaki Y; Sudo M; Ando S; Nakashima H; Saito T; Kaneoka H
Biochem Biophys Res Commun; 2014 May; 448(2):175-81. PubMed ID: 24780397
[TBL] [Abstract][Full Text] [Related]
8. Bezafibrate upregulates carnitine palmitoyltransferase II expression and promotes mitochondrial energy crisis dissipation in fibroblasts of patients with influenza-associated encephalopathy.
Yao M; Yao D; Yamaguchi M; Chida J; Yao D; Kido H
Mol Genet Metab; 2011 Nov; 104(3):265-72. PubMed ID: 21816645
[TBL] [Abstract][Full Text] [Related]
9. Analysis of carnitine esters by radio-high performance liquid chromatography in cultured skin fibroblasts from patients with mitochondrial fatty acid oxidation disorders.
Schmidt-Sommerfeld E; Bobrowski PJ; Penn D; Rhead WJ; Wanders RJ; Bennett MJ
Pediatr Res; 1998 Aug; 44(2):210-4. PubMed ID: 9702916
[TBL] [Abstract][Full Text] [Related]
10. Oxidation of fatty acids in cultured fibroblasts: a model system for the detection and study of defects in oxidation.
Saudubray JM; Coudé FX; Demaugre F; Johnson C; Gibson KM; Nyhan WL
Pediatr Res; 1982 Oct; 16(10):877-81. PubMed ID: 7145511
[TBL] [Abstract][Full Text] [Related]
11. Bezafibrate can be a new treatment option for mitochondrial fatty acid oxidation disorders: evaluation by in vitro probe acylcarnitine assay.
Yamaguchi S; Li H; Purevsuren J; Yamada K; Furui M; Takahashi T; Mushimoto Y; Kobayashi H; Hasegawa Y; Taketani T; Fukao T; Fukuda S
Mol Genet Metab; 2012 Sep; 107(1-2):87-91. PubMed ID: 22841441
[TBL] [Abstract][Full Text] [Related]
12. Genetic basis for correction of very-long-chain acyl-coenzyme A dehydrogenase deficiency by bezafibrate in patient fibroblasts: toward a genotype-based therapy.
Gobin-Limballe S; Djouadi F; Aubey F; Olpin S; Andresen BS; Yamaguchi S; Mandel H; Fukao T; Ruiter JP; Wanders RJ; McAndrew R; Kim JJ; Bastin J
Am J Hum Genet; 2007 Dec; 81(6):1133-43. PubMed ID: 17999356
[TBL] [Abstract][Full Text] [Related]
13. Carnitine-acylcarnitine translocase deficiency: Two neonatal cases with common splicing mutation and in vitro bezafibrate response.
Vatanavicharn N; Yamada K; Aoyama Y; Fukao T; Densupsoontorn N; Jirapinyo P; Sathienkijkanchai A; Yamaguchi S; Wasant P
Brain Dev; 2015 Aug; 37(7):698-703. PubMed ID: 25459972
[TBL] [Abstract][Full Text] [Related]
14. Mitochondrial trifunctional protein deficiency in human cultured fibroblasts: effects of bezafibrate.
Djouadi F; Habarou F; Le Bachelier C; Ferdinandusse S; Schlemmer D; Benoist JF; Boutron A; Andresen BS; Visser G; de Lonlay P; Olpin S; Fukao T; Yamaguchi S; Strauss AW; Wanders RJ; Bastin J
J Inherit Metab Dis; 2016 Jan; 39(1):47-58. PubMed ID: 26109258
[TBL] [Abstract][Full Text] [Related]
15. Analysis of mitochondrial fatty acid oxidation intermediates by tandem mass spectrometry from intact mitochondria prepared from homogenates of cultured fibroblasts, skeletal muscle cells, and fresh muscle.
Tyni T; Pourfarzam M; Turnbull DM
Pediatr Res; 2002 Jul; 52(1):64-70. PubMed ID: 12084849
[TBL] [Abstract][Full Text] [Related]
16. Inhibition of hepatic carnitine palmitoyl-transferase I (CPT IA) by valproyl-CoA as a possible mechanism of valproate-induced steatosis.
Aires CC; Ijlst L; Stet F; Prip-Buus C; de Almeida IT; Duran M; Wanders RJ; Silva MF
Biochem Pharmacol; 2010 Mar; 79(5):792-9. PubMed ID: 19854160
[TBL] [Abstract][Full Text] [Related]
17. A novel functional assay for simultaneous determination of total fatty acid beta-oxidation flux and acylcarnitine profiling in human skin fibroblasts using (2)H(31)-palmitate by isotope ratio mass spectrometry and electrospray tandem mass spectrometry.
Law LK; Tang NL; Hui J; Ho CS; Ruiter J; Fok TF; Wanders RJ; Lam CW
Clin Chim Acta; 2007 Jul; 382(1-2):25-30. PubMed ID: 17442290
[TBL] [Abstract][Full Text] [Related]
18. Bezafibrate in skeletal muscle fatty acid oxidation disorders: a randomized clinical trial.
Ørngreen MC; Madsen KL; Preisler N; Andersen G; Vissing J; Laforêt P
Neurology; 2014 Feb; 82(7):607-13. PubMed ID: 24453079
[TBL] [Abstract][Full Text] [Related]
19. Bezafibrate activation of PPAR drives disturbances in mitochondrial redox bioenergetics and decreases the viability of cells from patients with VLCAD deficiency.
Lund M; Andersen KG; Heaton R; Hargreaves IP; Gregersen N; Olsen RKJ
Biochim Biophys Acta Mol Basis Dis; 2021 Jun; 1867(6):166100. PubMed ID: 33549744
[TBL] [Abstract][Full Text] [Related]
20. Long-term follow-up of bezafibrate treatment in patients with the myopathic form of carnitine palmitoyltransferase 2 deficiency.
Bonnefont JP; Bastin J; Laforêt P; Aubey F; Mogenet A; Romano S; Ricquier D; Gobin-Limballe S; Vassault A; Behin A; Eymard B; Bresson JL; Djouadi F
Clin Pharmacol Ther; 2010 Jul; 88(1):101-8. PubMed ID: 20505667
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]