Artículo de revista
N-Acetylcysteine reduces skeletal muscles oxidative stress and improves grip strength in dysferlin-deficient Bla/J Mice
Fecha
2020Registro en:
Int. J. Mol. Sci. 2020, 21, 4293
10.3390/ijms21124293
Autor
García Campos, Paz
Báez Matus, Ximena
Jara Gutiérrez, Carlos
Paz Araos, Marilyn
Astorga, César
Cea, Luis A.
Pedroza Rodríguez, Viviana
Bevilacqua, Jorge Alfredo
Caviedes Fernández, Pablo
Cárdenas, Ana M.
Institución
Resumen
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 e cacy of antioxidant therapy remain unclear. Here,
we evaluated the e ect 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 a ecting 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.