Valproate Induces in Vitro Accumulation of Long-Chain Fatty Acylcarnitines |
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| Authors: | Margarida F. B. Silva Cornelis Jakobs Marinus Duran Isabel Tavares de Almeida Ronald J. A. Wanders |
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| Affiliation: | a Department of Clinical Chemistry and Paediatrics, University of Amsterdam, Academic Medical Centre, 1105 AZ, Amsterdam, The Netherlands;b Centro de Patogénese Molecular, Faculdade de Farmácia da Universidade de Lisboa, Av. Prof. Gama Pinto, 1649-003, Lisboa, Portugal;c Department of Clinical Chemistry, Free University Hospital, 1081 HV, Amsterdam, The Netherlands |
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| Abstract: | To elucidate the interference mechanisms of valproate (VPA) with mitochondrial fatty acid β-oxidation (FAO), the profile of acylcarnitine formation was studied in vitro. Human fibroblasts were incubated with 0.2 mmol/L [U-13C]palmitate, 0.4 mmol/L -carnitine, ± VPA (2 mmol/L) (96 h at 37°C). Acylcarnitines (AC) were analyzed by GC-CI-MS. VPA induced an impaired production of acetylcarnitine (C2) and an increase on long-chain AC (C10 to C16) both in control and in FAO-deficient cell lines (VLCAD, LCHAD, MTP). |
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| Keywords: | valproic acid acylcarnitine carnitine fatty acid β -oxidation long-chain fatty acids long-chain acylcarnitines valproate metabolism |
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