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

Summary Exposure to fibrates leads to normalization of fatty acid oxidation (FAO) in fibroblasts from patients with myopathic forms of CPT2 deficiency or VLCAD deficiency. Correction of FAO is related to a drug‐induced increase of residual enzyme activity, and this could provide a new treatment stra...

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Veröffentlicht in:	Journal of inherited metabolic disease 2006-04, Vol.29 (2-3), p.341-342
Hauptverfasser:	Djouadi, F., Aubey, F., Schlemmer, D., Gobin, S., Laforet, P., Wanders, R. J. A., Strauss, A. W., Bonnefont, J. P., Bastin, J.
Format:	Artikel
Sprache:	eng
Schlagworte:	Acyl-CoA Dehydrogenase, Long-Chain - biosynthesis Acyl-CoA Dehydrogenase, Long-Chain - deficiency Acyl-CoA Dehydrogenase, Long-Chain - genetics Bezafibrate - pharmacology Carnitine O-Palmitoyltransferase - biosynthesis Carnitine O-Palmitoyltransferase - deficiency Carnitine O-Palmitoyltransferase - genetics Cells, Cultured Clofibric Acid - pharmacology Clofibric Acid - therapeutic use Enzyme Induction - drug effects Fatty Acids - metabolism Fibroblasts - drug effects Fibroblasts - enzymology Humans Hypolipidemic Agents - pharmacology Hypolipidemic Agents - therapeutic use Life Sciences Lipid Metabolism, Inborn Errors - drug therapy Lipid Metabolism, Inborn Errors - genetics Lipid Metabolism, Inborn Errors - metabolism Mitochondria - drug effects Mitochondria - enzymology Oxidation-Reduction - drug effects PPAR gamma - agonists PPAR gamma - metabolism RNA, Messenger - biosynthesis
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Zusammenfassung:	Summary Exposure to fibrates leads to normalization of fatty acid oxidation (FAO) in fibroblasts from patients with myopathic forms of CPT2 deficiency or VLCAD deficiency. Correction of FAO is related to a drug‐induced increase of residual enzyme activity, and this could provide a new treatment strategy for these disorders.
ISSN:	0141-8955 1573-2665
DOI:	10.1007/s10545-006-0266-3