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Being a simple fatty acid, VPA is a substrate for the fatty acid \u03b2\u2010oxidation (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 \u03b2\u2010oxidation. 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\u2010induced steatosis and acute liver toxicity are two different concepts that will be emphasized.<\/jats:p>","DOI":"10.1007\/s10545-008-0841-x","type":"journal-article","created":{"date-parts":[[2008,4,7]],"date-time":"2008-04-07T01:39:39Z","timestamp":1207532379000},"page":"205-216","source":"Crossref","is-referenced-by-count":309,"title":["Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation: A review"],"prefix":"10.1002","volume":"31","author":[{"given":"M. F. B.","family":"Silva","sequence":"first","affiliation":[{"name":"Centro de Patog\u00e9nese Molecular\u2010UBMBE, iMed.UL Faculdade de Farm\u00e1cia da Universidade de Lisboa Av. Prof. Gama Pinto Lisboa 1649\u2013003 Portugal"}]},{"given":"C. C. 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