Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation: a review

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

doi:https://doi.org/10.1007/s10545-008-0841-x

Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation: a review

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

Research output: Contribution to journal › Article › Academic › peer-review

309 Citations (Scopus)

Abstract

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 beta-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 beta-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

Original language	English
Pages (from-to)	205-216
Journal	Journal of Inherited Metabolic Disease
Volume	31
Issue number	2
DOIs	https://doi.org/10.1007/s10545-008-0841-x
Publication status	Published - 2008

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https://doi.org/10.1007/s10545-008-0841-x

Cite this

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title = "Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation: a review",

abstract = "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 beta-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 beta-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",

author = "Silva, {M. F. B.} and Aires, {C. C. P.} and Luis, {P. B. M.} and Ruiter, {J. P. N.} and L. IJlst and M. Duran and Wanders, {R. J. A.} and {Tavares de Almeida}, I.",

year = "2008",

doi = "https://doi.org/10.1007/s10545-008-0841-x",

language = "English",

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journal = "Journal of Inherited Metabolic Disease",

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TY - JOUR

T1 - Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation: a review

AU - Silva, M. F. B.

AU - Aires, C. C. P.

AU - Luis, P. B. M.

AU - Ruiter, J. P. N.

AU - IJlst, L.

AU - Duran, M.

AU - Wanders, R. J. A.

AU - Tavares de Almeida, I.

PY - 2008

Y1 - 2008

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AB - 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 beta-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 beta-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

U2 - https://doi.org/10.1007/s10545-008-0841-x

DO - https://doi.org/10.1007/s10545-008-0841-x

M3 - Article

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SN - 0141-8955

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EP - 216

JO - Journal of Inherited Metabolic Disease

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