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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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author Silva, M. F. B.
author2 Aires, C. C. P.
Luis, P. B. M.
Ruiter, J. P. N.
IJist, L.
Duran, M.
Wanders, R. J. A.
de Almeida, I. Tavares
author2_role author
author
author
author
author
author
author
author_facet 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. Tavares
author_role author
contributor_name_str_mv Repositório Científico de Acesso Aberto da ULisboa
country_str PT
creators_json_txt [{\"Person.name\":\"Silva, M. F. B.\"},{\"Person.name\":\"Aires, C. C. P.\"},{\"Person.name\":\"Luis, P. B. M.\"},{\"Person.name\":\"Ruiter, J. P. N.\"},{\"Person.name\":\"IJist, L.\"},{\"Person.name\":\"Duran, M.\"},{\"Person.name\":\"Wanders, R. J. A.\"},{\"Person.name\":\"de Almeida, I. Tavares\"}]
datacite.contributors.contributor.contributorName.fl_str_mv Repositório Científico de Acesso Aberto da ULisboa
datacite.creators.creator.creatorName.fl_str_mv 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. Tavares
datacite.date.Accepted.fl_str_mv 2008-01-01T00:00:00Z
datacite.date.available.fl_str_mv 2015-12-30T10:18:36Z
datacite.date.embargoed.fl_str_mv 2015-12-30T10:18:36Z
datacite.rights.fl_str_mv http://purl.org/coar/access_right/c_14cb
datacite.subjects.subject.fl_str_mv Endocrinology & Metabolism
Genetics & Heredity
datacite.titles.title.fl_str_mv Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation
A review
dc.contributor.none.fl_str_mv Repositório Científico de Acesso Aberto da ULisboa
dc.creator.none.fl_str_mv 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. Tavares
dc.date.Accepted.fl_str_mv 2008-01-01T00:00:00Z
dc.date.available.fl_str_mv 2015-12-30T10:18:36Z
dc.date.embargoed.fl_str_mv 2015-12-30T10:18:36Z
dc.identifier.none.fl_str_mv http://hdl.handle.net/10451/21686
dc.language.none.fl_str_mv eng
dc.publisher.none.fl_str_mv DORDRECHT
dc.rights.none.fl_str_mv http://purl.org/coar/access_right/c_14cb
dc.subject.none.fl_str_mv Endocrinology & Metabolism
Genetics & Heredity
dc.title.fl_str_mv Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation
A review
dc.type.none.fl_str_mv http://purl.org/coar/resource_type/c_6501
description 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.
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person_str_mv 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. Tavares
publishDate 2008
publisher.none.fl_str_mv DORDRECHT
reponame_str Repositório da Universidade de Lisboa
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spelling engDORDRECHTValproic 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.Valproic acid metabolism and its effects on mitochondrial fatty acid oxidationA reviewSilva, 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. TavaresHostingInstitutionOrganizationalRepositório Científico de Acesso Aberto da ULisboae-mailmailto:repositorio@reitoria.ulisboa.ptrepositorio@reitoria.ulisboa.ptISBNIsPartOfChild Hlth, Great ODOIIsPartOfhttp://dx.doi.org/10.1007/s10545-008-0841-x2015-12-30T10:18:36Z20082008-01-01T00:00:00ZHandlehttp://hdl.handle.net/10451/21686http://purl.org/coar/access_right/c_14cbmetadata only accessEndocrinology & MetabolismGenetics & Heredityliteraturehttp://purl.org/coar/resource_type/c_6501journal article205216
spellingShingle Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation
Silva, M. F. B.
Endocrinology & Metabolism
Genetics & Heredity
status SINGLETON
subject.fl_str_mv Endocrinology & Metabolism
Genetics & Heredity
title Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation
title_full Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation
title_fullStr Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation
title_full_unstemmed Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation
title_short Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation
title_sort Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation
topic Endocrinology & Metabolism
Genetics & Heredity
topic_facet Endocrinology & Metabolism
Genetics & Heredity
url http://hdl.handle.net/10451/21686
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