Silva, M. F. B.Aires, C. C. P.Luis, P. B. M.Ruiter, J. P. N.IJist, L.Duran, M.Wanders, R. J. A.de Almeida, I. Tavares2015-12-302015-12-302008JOURNAL OF INHERITED METABOLIC DISEASE, APR 2008. - p. 205-216Child Hlth, Great Ohttp://hdl.handle.net/10451/21686Valproic 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.application/pdfengEndocrinology & MetabolismGenetics & Heredityjournal articlehttp://dx.doi.org/10.1007/s10545-008-0841-x