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Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation

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Resumo: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 P-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 P-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.
Autores principais:Silva, M. F. B.
Outros Autores:Aires, C. C. P.; Luis, P. B. M.; Ruiter, J. P. N.; IJist, L.; Duran, M.; Wanders, R. J. A.; de Almeida, I. Tavares
Assunto:Endocrinology & Metabolism Genetics & Heredity
Ano:2008
País:Portugal
Tipo de documento:artigo
Tipo de acesso:acesso a metadados
Instituição associada:Universidade de Lisboa
Idioma:inglês
Origem:Repositório da Universidade de Lisboa

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