Urinary biomarkers of oxidative damage in maple syrup urine disease : the l-carnitine role

Detalhes bibliográficos
Autor(a) principal: Guerreiro, Gilian Batista Balbueno
Data de Publicação: 2014
Tipo de documento: Trabalho de conclusão de curso
Idioma: eng
Título da fonte: Repositório Institucional da UFRGS
Texto Completo: http://hdl.handle.net/10183/183417
Resumo: Maple Syrup Urine Disease (MSUD) is a disorder of branched-chain amino acids (BCAA). The defect in the branched-chain α-keto acid dehydrogenase complex activity lead to an accumulation of these compounds and their corresponding α-keto-acids and α-hydroxy-acids. Studies have shown that oxidative stress may be involved in neuropathology of MSUD. L-carnitine (L-car) have an important role as antioxidant through reducing and scavenging free radicals formation and by enhancing the activity of antioxidant enzymes. Our study evaluated the oxidative stress parameters, dityrosine, isoprostanes and antioxidant capacity, in urine of MSUD patients on proteinrestricted diet supplemented or not with L-car capsules at a dose of 50 mg kg-1 day-1. We also determined the BCAAs, α-keto acids and α-hydroxy levels in urine and quantified the free L-car levels. Firstly, we found a deficiency of carnitine in patients before the L-car supplementation. Significant increases of di-tyrosine and isoprostanes, as well as reduced antioxidant capacity were observed in urine of MSUD patients before the treatment with L-car. The L-car supplementation was able to reduce the di-tyrosine and isoprostanes levels, as well as to increase the antioxidant capacity. We also quantified the BCAAs and metabolites in urine from these patients, detecting a significantly increase of leucine, isoleucine, α-ketoisocaproic acid, α- hydroxyisocaproate and α-hydroxy-B-methylvalerate after 2 months of treatment, compared to control group. In conclusion, our results suggest that L-car may have additional beneficial effects in the treatment of MSUD, not only by preventing oxidative damage, but also by increasing the excretion of MSUD metabolites, reducing the toxic effects caused by their accumulation.
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spelling Guerreiro, Gilian Batista BalbuenoVargas, Carmen ReglaMescka, Caroline Paula2018-10-16T02:43:14Z2014http://hdl.handle.net/10183/183417000937533Maple Syrup Urine Disease (MSUD) is a disorder of branched-chain amino acids (BCAA). The defect in the branched-chain α-keto acid dehydrogenase complex activity lead to an accumulation of these compounds and their corresponding α-keto-acids and α-hydroxy-acids. Studies have shown that oxidative stress may be involved in neuropathology of MSUD. L-carnitine (L-car) have an important role as antioxidant through reducing and scavenging free radicals formation and by enhancing the activity of antioxidant enzymes. Our study evaluated the oxidative stress parameters, dityrosine, isoprostanes and antioxidant capacity, in urine of MSUD patients on proteinrestricted diet supplemented or not with L-car capsules at a dose of 50 mg kg-1 day-1. We also determined the BCAAs, α-keto acids and α-hydroxy levels in urine and quantified the free L-car levels. Firstly, we found a deficiency of carnitine in patients before the L-car supplementation. Significant increases of di-tyrosine and isoprostanes, as well as reduced antioxidant capacity were observed in urine of MSUD patients before the treatment with L-car. The L-car supplementation was able to reduce the di-tyrosine and isoprostanes levels, as well as to increase the antioxidant capacity. We also quantified the BCAAs and metabolites in urine from these patients, detecting a significantly increase of leucine, isoleucine, α-ketoisocaproic acid, α- hydroxyisocaproate and α-hydroxy-B-methylvalerate after 2 months of treatment, compared to control group. In conclusion, our results suggest that L-car may have additional beneficial effects in the treatment of MSUD, not only by preventing oxidative damage, but also by increasing the excretion of MSUD metabolites, reducing the toxic effects caused by their accumulation.application/pdfengBiomarcadoresDoença da urina de xarope de bordoEstresse oxidativoMaple syrup urine diseaseL-carnitineOxidative stressAntioxidantUrinary biomarkers of oxidative damage in maple syrup urine disease : the l-carnitine roleinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/bachelorThesisUniversidade Federal do Rio Grande do SulFaculdade de FarmáciaPorto Alegre, BR-RS2014Farmáciagraduaçãoinfo:eu-repo/semantics/openAccessreponame:Repositório Institucional da UFRGSinstname:Universidade Federal do Rio Grande do Sul (UFRGS)instacron:UFRGSORIGINAL000937533.pdfTexto completo (inglês)application/pdf1026856http://www.lume.ufrgs.br/bitstream/10183/183417/1/000937533.pdf8668fbf39913a4ccdfe756d4850d3621MD51TEXT000937533.pdf.txt000937533.pdf.txtExtracted Texttext/plain62512http://www.lume.ufrgs.br/bitstream/10183/183417/2/000937533.pdf.txt13713664dee95cd29382623d91da218eMD52THUMBNAIL000937533.pdf.jpg000937533.pdf.jpgGenerated Thumbnailimage/jpeg971http://www.lume.ufrgs.br/bitstream/10183/183417/3/000937533.pdf.jpg8b6277a5c1759af3439b1133c1931d58MD5310183/1834172018-10-17 02:37:41.359293oai:www.lume.ufrgs.br:10183/183417Repositório de PublicaçõesPUBhttps://lume.ufrgs.br/oai/requestopendoar:2018-10-17T05:37:41Repositório Institucional da UFRGS - Universidade Federal do Rio Grande do Sul (UFRGS)false
dc.title.pt_BR.fl_str_mv Urinary biomarkers of oxidative damage in maple syrup urine disease : the l-carnitine role
title Urinary biomarkers of oxidative damage in maple syrup urine disease : the l-carnitine role
spellingShingle Urinary biomarkers of oxidative damage in maple syrup urine disease : the l-carnitine role
Guerreiro, Gilian Batista Balbueno
Biomarcadores
Doença da urina de xarope de bordo
Estresse oxidativo
Maple syrup urine disease
L-carnitine
Oxidative stress
Antioxidant
title_short Urinary biomarkers of oxidative damage in maple syrup urine disease : the l-carnitine role
title_full Urinary biomarkers of oxidative damage in maple syrup urine disease : the l-carnitine role
title_fullStr Urinary biomarkers of oxidative damage in maple syrup urine disease : the l-carnitine role
title_full_unstemmed Urinary biomarkers of oxidative damage in maple syrup urine disease : the l-carnitine role
title_sort Urinary biomarkers of oxidative damage in maple syrup urine disease : the l-carnitine role
author Guerreiro, Gilian Batista Balbueno
author_facet Guerreiro, Gilian Batista Balbueno
author_role author
dc.contributor.author.fl_str_mv Guerreiro, Gilian Batista Balbueno
dc.contributor.advisor1.fl_str_mv Vargas, Carmen Regla
dc.contributor.advisor-co1.fl_str_mv Mescka, Caroline Paula
contributor_str_mv Vargas, Carmen Regla
Mescka, Caroline Paula
dc.subject.por.fl_str_mv Biomarcadores
Doença da urina de xarope de bordo
Estresse oxidativo
topic Biomarcadores
Doença da urina de xarope de bordo
Estresse oxidativo
Maple syrup urine disease
L-carnitine
Oxidative stress
Antioxidant
dc.subject.eng.fl_str_mv Maple syrup urine disease
L-carnitine
Oxidative stress
Antioxidant
description Maple Syrup Urine Disease (MSUD) is a disorder of branched-chain amino acids (BCAA). The defect in the branched-chain α-keto acid dehydrogenase complex activity lead to an accumulation of these compounds and their corresponding α-keto-acids and α-hydroxy-acids. Studies have shown that oxidative stress may be involved in neuropathology of MSUD. L-carnitine (L-car) have an important role as antioxidant through reducing and scavenging free radicals formation and by enhancing the activity of antioxidant enzymes. Our study evaluated the oxidative stress parameters, dityrosine, isoprostanes and antioxidant capacity, in urine of MSUD patients on proteinrestricted diet supplemented or not with L-car capsules at a dose of 50 mg kg-1 day-1. We also determined the BCAAs, α-keto acids and α-hydroxy levels in urine and quantified the free L-car levels. Firstly, we found a deficiency of carnitine in patients before the L-car supplementation. Significant increases of di-tyrosine and isoprostanes, as well as reduced antioxidant capacity were observed in urine of MSUD patients before the treatment with L-car. The L-car supplementation was able to reduce the di-tyrosine and isoprostanes levels, as well as to increase the antioxidant capacity. We also quantified the BCAAs and metabolites in urine from these patients, detecting a significantly increase of leucine, isoleucine, α-ketoisocaproic acid, α- hydroxyisocaproate and α-hydroxy-B-methylvalerate after 2 months of treatment, compared to control group. In conclusion, our results suggest that L-car may have additional beneficial effects in the treatment of MSUD, not only by preventing oxidative damage, but also by increasing the excretion of MSUD metabolites, reducing the toxic effects caused by their accumulation.
publishDate 2014
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