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Erschienen in: Journal of Inherited Metabolic Disease 2/2008

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.
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Metadaten
Titel
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
Publikationsdatum
01.04.2008
Verlag
Springer Netherlands
Erschienen in
Journal of Inherited Metabolic Disease / Ausgabe 2/2008
Print ISSN: 0141-8955
Elektronische ISSN: 1573-2665
DOI
https://doi.org/10.1007/s10545-008-0841-x

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