Coenzyme Q10 in the Treatment of Mitochondrial Disease

Detalhes bibliográficos
Autor(a) principal: Neergheen,Viruna
Data de Publicação: 2017
Outros Autores: Chalasani,Annapurna, Wainwright,Luke, Yubero,Delia, Montero,Raquel, Artuch,Rafael, Hargreaves,Iain
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Journal of Inborn Errors of Metabolism and Screening
Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S2326-45942017000100801
Resumo: Abstract Currently, there is a paucity of available treatment strategies for oxidative phosphorylation disorders. Coenzyme Q10 (CoQ10) and related synthetic quinones are the only agents to date that have proven to be beneficial in the treatment of these heterogeneous disorders. The therapeutic efficacy of CoQ10 is not restricted to patients with an underlying CoQ10 deficiency and is thought to result from its ability to restore electron flow in the mitochondrial respiratory chain (MRC) as well as to increase the cellular antioxidant capacity. At present, however, there is no consensus on the appropriate dosage or therapeutic plasma level of CoQ10, and this information will be required before CoQ10 can be utilized effectively in the treatment of mitochondrial disease. The following review will outline our current knowledge on the use of CoQ10 in the treatment of MRC disorders and primary CoQ10 deficiencies.
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spelling Coenzyme Q10 in the Treatment of Mitochondrial Diseasecoenzyme Q10mitochondrial respiratory chain (MRC)oxidative stressantioxidantAbstract Currently, there is a paucity of available treatment strategies for oxidative phosphorylation disorders. Coenzyme Q10 (CoQ10) and related synthetic quinones are the only agents to date that have proven to be beneficial in the treatment of these heterogeneous disorders. The therapeutic efficacy of CoQ10 is not restricted to patients with an underlying CoQ10 deficiency and is thought to result from its ability to restore electron flow in the mitochondrial respiratory chain (MRC) as well as to increase the cellular antioxidant capacity. At present, however, there is no consensus on the appropriate dosage or therapeutic plasma level of CoQ10, and this information will be required before CoQ10 can be utilized effectively in the treatment of mitochondrial disease. The following review will outline our current knowledge on the use of CoQ10 in the treatment of MRC disorders and primary CoQ10 deficiencies.Latin American Society Inborn Errors and Neonatal Screening (SLEIMPN); Instituto Genética para Todos (IGPT)2017-01-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S2326-45942017000100801Journal of Inborn Errors of Metabolism and Screening v.5 2017reponame:Journal of Inborn Errors of Metabolism and Screeninginstname:Instituto Genética para Todos (IGPT)instacron:IGPT10.1177/2326409817707771info:eu-repo/semantics/openAccessNeergheen,VirunaChalasani,AnnapurnaWainwright,LukeYubero,DeliaMontero,RaquelArtuch,RafaelHargreaves,Iaineng2019-05-14T00:00:00Zoai:scielo:S2326-45942017000100801Revistahttp://jiems-journal.org/ONGhttps://old.scielo.br/oai/scielo-oai.phpjiems@jiems-journal.org||rgiugliani@hcpa.edu.br2326-45942326-4594opendoar:2019-05-14T00:00Journal of Inborn Errors of Metabolism and Screening - Instituto Genética para Todos (IGPT)false
dc.title.none.fl_str_mv Coenzyme Q10 in the Treatment of Mitochondrial Disease
title Coenzyme Q10 in the Treatment of Mitochondrial Disease
spellingShingle Coenzyme Q10 in the Treatment of Mitochondrial Disease
Neergheen,Viruna
coenzyme Q10
mitochondrial respiratory chain (MRC)
oxidative stress
antioxidant
title_short Coenzyme Q10 in the Treatment of Mitochondrial Disease
title_full Coenzyme Q10 in the Treatment of Mitochondrial Disease
title_fullStr Coenzyme Q10 in the Treatment of Mitochondrial Disease
title_full_unstemmed Coenzyme Q10 in the Treatment of Mitochondrial Disease
title_sort Coenzyme Q10 in the Treatment of Mitochondrial Disease
author Neergheen,Viruna
author_facet Neergheen,Viruna
Chalasani,Annapurna
Wainwright,Luke
Yubero,Delia
Montero,Raquel
Artuch,Rafael
Hargreaves,Iain
author_role author
author2 Chalasani,Annapurna
Wainwright,Luke
Yubero,Delia
Montero,Raquel
Artuch,Rafael
Hargreaves,Iain
author2_role author
author
author
author
author
author
dc.contributor.author.fl_str_mv Neergheen,Viruna
Chalasani,Annapurna
Wainwright,Luke
Yubero,Delia
Montero,Raquel
Artuch,Rafael
Hargreaves,Iain
dc.subject.por.fl_str_mv coenzyme Q10
mitochondrial respiratory chain (MRC)
oxidative stress
antioxidant
topic coenzyme Q10
mitochondrial respiratory chain (MRC)
oxidative stress
antioxidant
description Abstract Currently, there is a paucity of available treatment strategies for oxidative phosphorylation disorders. Coenzyme Q10 (CoQ10) and related synthetic quinones are the only agents to date that have proven to be beneficial in the treatment of these heterogeneous disorders. The therapeutic efficacy of CoQ10 is not restricted to patients with an underlying CoQ10 deficiency and is thought to result from its ability to restore electron flow in the mitochondrial respiratory chain (MRC) as well as to increase the cellular antioxidant capacity. At present, however, there is no consensus on the appropriate dosage or therapeutic plasma level of CoQ10, and this information will be required before CoQ10 can be utilized effectively in the treatment of mitochondrial disease. The following review will outline our current knowledge on the use of CoQ10 in the treatment of MRC disorders and primary CoQ10 deficiencies.
publishDate 2017
dc.date.none.fl_str_mv 2017-01-01
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://old.scielo.br/scielo.php?script=sci_arttext&pid=S2326-45942017000100801
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S2326-45942017000100801
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 10.1177/2326409817707771
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv text/html
dc.publisher.none.fl_str_mv Latin American Society Inborn Errors and Neonatal Screening (SLEIMPN); Instituto Genética para Todos (IGPT)
publisher.none.fl_str_mv Latin American Society Inborn Errors and Neonatal Screening (SLEIMPN); Instituto Genética para Todos (IGPT)
dc.source.none.fl_str_mv Journal of Inborn Errors of Metabolism and Screening v.5 2017
reponame:Journal of Inborn Errors of Metabolism and Screening
instname:Instituto Genética para Todos (IGPT)
instacron:IGPT
instname_str Instituto Genética para Todos (IGPT)
instacron_str IGPT
institution IGPT
reponame_str Journal of Inborn Errors of Metabolism and Screening
collection Journal of Inborn Errors of Metabolism and Screening
repository.name.fl_str_mv Journal of Inborn Errors of Metabolism and Screening - Instituto Genética para Todos (IGPT)
repository.mail.fl_str_mv jiems@jiems-journal.org||rgiugliani@hcpa.edu.br
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