Potential of fibrates in the treatment of fatty acid oxidation disorders: Revival of classical drugs?

Journal of Inherited Metabolic Disease

Volumn 29, Issue 2-3, 2006, Pages 341-342

  Djouadi, F ^a   Aubey, F ^a   Schlemmer, D ^a   Gobin, S ^e   Laforet, P ^b   Wanders, R J A ^c   Strauss, A W ^d   Bonnefont, J P ^a   Bastin, J ^a  

^a HÔPITAL NECKER ENFANTS MALADES   (France)

^b PITIÉ SALPÊTRIÈRE HOSPITAL   (France)

^c ACADEMIC MEDICAL CENTER   (Netherlands)

^d MONROE CARELL JR CHILDREN S HOSPITAL AT VANDERBILT   (United States)

^e NONE

Author keywords

[No Author keywords available]

Indexed keywords

ACYLCARNITINE; BEZAFIBRATE; FIBRIC ACID DERIVATIVE; LONG CHAIN ACYL COENZYME A DEHYDROGENASE; MESSENGER RNA; PALMITIC ACID; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR DELTA;

ARTICLE; DOSE RESPONSE; DRUG EFFICACY; DRUG MECHANISM; DRUG POTENCY; ENZYME ACTIVITY; ENZYME DEFICIENCY; FATTY ACID METABOLISM; FATTY ACID OXIDATION; FIBROBLAST; GENE EXPRESSION; GENE MUTATION; HUMAN; HUMAN CELL; HYPOTHESIS; MUSCLE CELL; MYOBLAST; MYOTUBE; PROTEIN SYNTHESIS; QUANTITATIVE ANALYSIS; REVERSE TRANSCRIPTION POLYMERASE CHAIN REACTION; TANDEM MASS SPECTROMETRY;

ACYL-COA DEHYDROGENASE, LONG-CHAIN; ANTILIPEMIC AGENTS; BEZAFIBRATE; CARNITINE O-PALMITOYLTRANSFERASE; CELLS, CULTURED; CLOFIBRIC ACID; ENZYME INDUCTION; FATTY ACIDS; FIBROBLASTS; HUMANS; HYPOLIPIDEMIC AGENTS; LIPID METABOLISM, INBORN ERRORS; MITOCHONDRIA; OXIDATION-REDUCTION; PPAR GAMMA; RNA, MESSENGER;

EID: 33745059957     PISSN: 01418955     EISSN: 15732665     Source Type: Journal    
DOI: 10.1007/s10545-006-0266-3     Document Type: Article

References (6)

1. Berger J, Moller DE (2002) The mechanisms of action of PPARs. Annu Rev Med 53: 409-435.
2. Djouadi F, Bonnefont JP, Thuillier L, et al (2003) Correction of fatty acid oxidation in carnitine palmitoyl transferase II-deficient cultured skin fibroblasts by bezafibrate. Pediatr Res 54: 446-451.
3. Djouadi F, Aubey F, Schlemmer D, Bastin J (2005a) Peroxisome proliferator activated receptor δ (PPARδ) agonist but not PPARα corrects carnitine palmitoyl transferase 2 deficiency in human muscle cells. J Clin Endocrinol Metab 90: 1791-1797.
4. Djouadi F, Aubey F, Schlemmer D, et al (2005b) 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 (2005) Hum Mol Genet 14: 2695-2703.
5. Gregersen N, Bross P, Andresen BS (2004) Genetic defects in fatty acid beta-oxidation and acyl-CoA dehydrogenases. Molecular pathogenesis and genotype-phenotype relationships. Eur J Biochem 271: 470-482.
6. Rinaldo P, Matern D, Bennett MJ (2002) Fatty acid oxidation disorders. Annu Rev Physiol 64: 477-502.