N-Acetylcysteine Reduces Skeletal Muscles Oxidative Stress and Improves Grip Strength in Dysferlin-Deficient Bla/J Mice
Author
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García Campos, Paz
Author
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Báez Matus, Ximena
Author
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Jara Gutiérrez, Carlos
Author
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Paz Araos, Marilyn
Author
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Astorga, César
Author
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Cea, Luis A.
Author
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Rodríguez, Viviana
Author
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Bevilacqua, Jorge A.
Author
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Caviedes, Pablo
Author
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Cárdenas, Ana M.
Admission date
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2020-10-15T23:31:04Z
Available date
dc.date.available
2020-10-15T23:31:04Z
Publication date
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2020
Cita de ítem
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Int. J. Mol. Sci. 2020, 21, 4293
es_ES
Identifier
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10.3390/ijms21124293
Identifier
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https://repositorio.uchile.cl/handle/2250/177178
Abstract
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Dysferlinopathy 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
Patrocinador
dc.description.sponsorship
Comision 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 Turismo