{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T02:07:38Z","timestamp":1790474858342,"version":"4.1.0"},"reference-count":79,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2020,6,16]],"date-time":"2020-06-16T00:00:00Z","timestamp":1592265600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100002850","name":"Fondo Nacional de Desarrollo Cient\u00edfico y Tecnol\u00f3gico","doi-asserted-by":"publisher","award":["1160495 (to AMC), 1151383 (to JAB)"],"award-info":[{"award-number":["1160495 (to AMC), 1151383 (to JAB)"]}],"id":[{"id":"10.13039\/501100002850","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>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 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\u2019s 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.<\/jats:p>","DOI":"10.3390\/ijms21124293","type":"journal-article","created":{"date-parts":[[2020,6,16]],"date-time":"2020-06-16T13:20:43Z","timestamp":1592313643000},"page":"4293","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["N-Acetylcysteine Reduces Skeletal Muscles Oxidative Stress and Improves Grip Strength in Dysferlin-Deficient Bla\/J Mice"],"prefix":"10.3390","volume":"21","author":[{"given":"Paz","family":"Garc\u00eda-Campos","sequence":"first","affiliation":[{"name":"Centro Interdisciplinario de Neurociencia de Valpara\u00edso (CNIV), Facultad de Ciencias, Universidad de Valpara\u00edso, Valpara\u00edso 2360102, Chile"},{"name":"Centro de Investigaciones Biom\u00e9dicas (CIB), Facultad de Medicina, Universidad de Valpara\u00edso, Valpara\u00edso 2520000, Chile"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4176-0239","authenticated-orcid":false,"given":"Ximena","family":"B\u00e1ez-Matus","sequence":"additional","affiliation":[{"name":"Centro Interdisciplinario de Neurociencia de Valpara\u00edso (CNIV), Facultad de Ciencias, Universidad de Valpara\u00edso, Valpara\u00edso 2360102, Chile"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos","family":"Jara-Guti\u00e9rrez","sequence":"additional","affiliation":[{"name":"Centro de Investigaciones Biom\u00e9dicas (CIB), Facultad de Medicina, Universidad de Valpara\u00edso, Valpara\u00edso 2520000, Chile"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marilyn","family":"Paz-Araos","sequence":"additional","affiliation":[{"name":"Centro de Investigaciones Biom\u00e9dicas (CIB), Facultad de Medicina, Universidad de Valpara\u00edso, Valpara\u00edso 2520000, Chile"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u00e9sar","family":"Astorga","sequence":"additional","affiliation":[{"name":"Programa de Farmacolog\u00eda Molecular y Cl\u00ednica, ICBM, Facultad de Medicina, and Centro de Biotecnolog\u00eda y Bioingenier\u00eda (CeBiB), Departamento de Ingenier\u00eda Qu\u00edmica, Biotecnolog\u00eda y Materiales, Facultad de Ciencias F\u00edsicas y Matem\u00e1ticas, Universidad de Chile, Santiago 8389100, Chile"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luis A.","family":"Cea","sequence":"additional","affiliation":[{"name":"Instituto de Ciencias Biom\u00e9dicas, Facultad de Ciencias de la Salud, Universidad Aut\u00f3noma de Chile, Santiago 8910060, Chile"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Viviana","family":"Rodr\u00edguez","sequence":"additional","affiliation":[{"name":"Escuela Psicolog\u00eda, Facultad de Ciencias Sociales, Universidad de Valpara\u00edso, Valpara\u00edso 2340000, Chile"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jorge A.","family":"Bevilacqua","sequence":"additional","affiliation":[{"name":"Departamento de Neurolog\u00eda y Neurocirug\u00eda, Hospital Cl\u00ednico Universidad de Chile and Departamento de Anatom\u00eda y Medicina Legal, Facultad de Medicina, Universidad de Chile, Santiago 8389100, Chile"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4462-7422","authenticated-orcid":false,"given":"Pablo","family":"Caviedes","sequence":"additional","affiliation":[{"name":"Programa de Farmacolog\u00eda Molecular y Cl\u00ednica, ICBM, Facultad de Medicina, and Centro de Biotecnolog\u00eda y Bioingenier\u00eda (CeBiB), Departamento de Ingenier\u00eda Qu\u00edmica, Biotecnolog\u00eda y Materiales, Facultad de Ciencias F\u00edsicas y Matem\u00e1ticas, Universidad de Chile, Santiago 8389100, Chile"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8747-4916","authenticated-orcid":false,"given":"Ana M.","family":"C\u00e1rdenas","sequence":"additional","affiliation":[{"name":"Centro Interdisciplinario de Neurociencia de Valpara\u00edso (CNIV), Facultad de Ciencias, Universidad de Valpara\u00edso, Valpara\u00edso 2360102, Chile"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,6,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1007\/s10974-014-9380-2","article-title":"Oxidative stress in muscular dystrophy: From generic evidence to specific sources and targets","volume":"35","author":"Canton","year":"2014","journal-title":"J. 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