Erschienen in:
01.04.2008 | SSIEM Symposium 2007
Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation: A review
verfasst von:
M. F. B. Silva, C. C. P. Aires, P. B. M. Luis, J. P. N. Ruiter, L. IJlst, M. Duran, R. J. A. Wanders, I. Tavares de Almeida
Erschienen in:
Journal of Inherited Metabolic Disease
|
Ausgabe 2/2008
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Summary
Valproic acid (VPA; 2-n-propylpentanoic acid) is widely used as a major drug in the treatment of epilepsy and in the control of several types of seizures. Being a simple fatty acid, VPA is a substrate for the fatty acid β-oxidation (FAO) pathway, which takes place primarily in mitochondria. The toxicity of valproate has long been considered to be due primarily to its interference with mitochondrial β-oxidation. The metabolism of the drug, its effects on enzymes of FAO and their cofactors such as CoA and/or carnitine will be reviewed. The cumulative consequences of VPA therapy in inborn errors of metabolism (IEMs) and the importance of recognizing an underlying IEM in cases of VPA-induced steatosis and acute liver toxicity are two different concepts that will be emphasized.