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dc.contributor.authorGarcía-Campos P.
dc.contributor.authorBáez-Matus X.
dc.contributor.authorJara-Gutiérrez C.
dc.contributor.authorPaz-Araos M.
dc.contributor.authorAstorga C.
dc.contributor.authorCea L.A.
dc.contributor.authorRodríguez V.
dc.contributor.authorBevilacqua J.A.
dc.contributor.authorCaviedes P.
dc.contributor.authorCárdenas A.M.
dc.date.accessioned2020-09-02T22:18:46Z
dc.date.available2020-09-02T22:18:46Z
dc.date.issued2020
dc.identifier10.3390/ijms21124293
dc.identifier.citation21, 12, 1-17
dc.identifier.issn16616596
dc.identifier.urihttps://hdl.handle.net/20.500.12728/4617
dc.descriptionDysferlinopathy 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 agent N-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. © 2020 by the authors. Licensee MDPI, Basel, Switzerland.
dc.language.isoen
dc.publisherMDPI AG
dc.subjectDysferlin
dc.subjectDysferlinopathy
dc.subjectN-acetylcysteine
dc.subjectOxidative stress
dc.subjectacetylcysteine
dc.subjectamino acid
dc.subjectarginine
dc.subjectcarbonyl iron
dc.subjectcatalase
dc.subjectcytochrome
dc.subjectdysferlin
dc.subjecthydrogen
dc.subjecthydrogen peroxide
dc.subjectimmunoglobulin enhancer binding protein
dc.subjectlipid
dc.subjectlysine
dc.subjectmalonaldehyde
dc.subjectproline
dc.subjectprotein
dc.subjectreduced nicotinamide adenine dinucleotide phosphate oxidase 2
dc.subjectserum albumin
dc.subjectsuperoxide dismutase
dc.subjectthiobarbituric acid reactive substance
dc.subjectthreonine
dc.subjecttrolox C
dc.subjecttyrosine
dc.subjectxanthine
dc.subjectxanthine oxidase
dc.subjectanimal experiment
dc.subjectanimal model
dc.subjectanimal tissue
dc.subjectantioxidant activity
dc.subjectantioxidant assay
dc.subjectArticle
dc.subjectbody mass
dc.subjectbody weight loss
dc.subjectcomparative study
dc.subjectcontrolled study
dc.subjectdiet supplementation
dc.subjectdysferlinopathy
dc.subjectenzyme activity
dc.subjectfatigue
dc.subjectgastrocnemius muscle
dc.subjectgene mutation
dc.subjectgenotyping technique
dc.subjectgrip strength
dc.subjectkinetics
dc.subjectKondzielas inverted screen test
dc.subjectlatency to fall
dc.subjectlipid peroxidation
dc.subjectlipid peroxidation assay
dc.subjectmouse
dc.subjectmuscle atrophy
dc.subjectmuscle mass
dc.subjectmuscular dystrophy
dc.subjectnonhuman
dc.subjectoxidative stress
dc.subjectpolymerase chain reaction
dc.subjectprotein carbonylation
dc.subjectquadriceps femoris muscle
dc.subjectretroposon
dc.subjectskeletal muscle
dc.subjecttransient absorption spectroscopy
dc.subjectultraviolet spectrophotometry
dc.titleN-acetylcysteine reduces skeletal muscles oxidative stress and improves grip strength in dysferlin-deficient BLA/J mice
dc.typeArticle


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