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Authordc.contributor.authorGarcía Campos, Paz 
Authordc.contributor.authorBáez Matus, Ximena 
Authordc.contributor.authorJara Gutiérrez, Carlos 
Authordc.contributor.authorPaz Araos, Marilyn 
Authordc.contributor.authorAstorga, César 
Authordc.contributor.authorCea, Luis A. 
Authordc.contributor.authorRodríguez, Viviana 
Authordc.contributor.authorBevilacqua, Jorge A. 
Authordc.contributor.authorCaviedes, Pablo 
Authordc.contributor.authorCárdenas, Ana M. 
Admission datedc.date.accessioned2020-10-15T23:31:04Z
Available datedc.date.available2020-10-15T23:31:04Z
Publication datedc.date.issued2020
Cita de ítemdc.identifier.citationInt. J. Mol. Sci. 2020, 21, 4293es_ES
Identifierdc.identifier.other10.3390/ijms21124293
Identifierdc.identifier.urihttp://repositorio.uchile.cl/handle/2250/177178
Abstractdc.description.abstractDysferlinopathy is an autosomal recessive muscular dystrophy resulting from mutations in the dysferlin gene. Absence of dysferlin in the sarcolemma and progressive muscle wasting are hallmarks of this disease. Signs of oxidative stress have been observed in skeletal muscles of dysferlinopathy patients, as well as in dysferlin-deficient mice. However, the contribution of the redox imbalance to this pathology and the efficacy of antioxidant therapy remain unclear. Here, we evaluated the effect of 10 weeks diet supplementation with the antioxidant agentN-acetylcysteine (NAC, 1%) on measurements of oxidative damage, antioxidant enzymes, grip strength and body mass in 6 months-old dysferlin-deficient Bla/J mice and wild-type (WT) C57 BL/6 mice. We found that quadriceps and gastrocnemius muscles of Bla/J mice exhibit high levels of lipid peroxidation, protein carbonyls and superoxide dismutase and catalase activities, which were significantly reduced by NAC supplementation. By using the Kondziela's inverted screen test, we further demonstrated that NAC improved grip strength in dysferlin deficient animals, as compared with non-treated Bla/J mice, without affecting body mass. Together, these results indicate that this antioxidant agent improves skeletal muscle oxidative balance, as well as muscle strength and/or resistance to fatigue in dysferlin-deficient animals.es_ES
Patrocinadordc.description.sponsorshipComision Nacional de Investigacion Cientifica y Tecnologica (CONICYT) CONICYT FONDECYT 1160495 1151383 ICM-ANID, Chile P09-022-F Comision Nacional de Investigacion Cientifica y Tecnologica (CONICYT) FB0001 Millennium Scientific Initiative of the Ministerio de Economia, Fomento y Turismoes_ES
Lenguagedc.language.isoenes_ES
Publisherdc.publisherMDPIes_ES
Type of licensedc.rightsAttribution-NonCommercial-NoDerivs 3.0 Chile*
Link to Licensedc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/3.0/cl/*
Sourcedc.sourceInternational Journal o f Molecular Scienceses_ES
Keywordsdc.subjectDysferlines_ES
Keywordsdc.subjectDysferlinopathyes_ES
Keywordsdc.subjectOxidative stresses_ES
Keywordsdc.subjectN-acetylcysteinees_ES
Títulodc.titleN-Acetylcysteine Reduces Skeletal Muscles Oxidative Stress and Improves Grip Strength in Dysferlin-Deficient Bla/J Micees_ES
Document typedc.typeArtículo de revistaes_ES
dcterms.accessRightsdcterms.accessRightsAcceso Abierto
Catalogueruchile.catalogadorlajes_ES
Indexationuchile.indexArtículo de publicación ISIes_ES


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Attribution-NonCommercial-NoDerivs 3.0 Chile
Except where otherwise noted, this item's license is described as Attribution-NonCommercial-NoDerivs 3.0 Chile