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Using transcriptome and proteome profiling as well as functional assays, we previously demonstrated significant metabolic impairment in skeletal muscle from LAMA2-CMD patients and mouse models. Reactive oxygen species (ROS) increase when oxygen homeostasis is not maintained and, here, we investigate whether oxidative stress indeed is involved in the pathogenesis of LAMA2-CMD. We also analyze the effects of two antioxidant molecules, N-acetyl-L-cysteine (NAC) and vitamin E, on disease progression in the dy2J\/dy2J mouse model of LAMA2-CMD. We demonstrate increased ROS levels in LAMA2-CMD mouse and patient skeletal muscle. Furthermore, NAC treatment (150 mg\/kg IP for 6 days\/week for 3 weeks) led to muscle force loss prevention, reduced central nucleation and decreased the occurrence of apoptosis, inflammation, fibrosis and oxidative stress in LAMA2-CMD muscle. In addition, vitamin E (40 mg\/kg oral gavage for 6 days\/week for 2 weeks) improved morphological features and reduced inflammation and ROS levels in dy2J\/dy2J skeletal muscle. We suggest that NAC and to some extent vitamin E might be potential future supportive treatments for LAMA2-CMD as they improve numerous pathological hallmarks of LAMA2-CMD.<\/jats:p>","DOI":"10.3390\/antiox9030244","type":"journal-article","created":{"date-parts":[[2020,3,19]],"date-time":"2020-03-19T03:54:14Z","timestamp":1584590054000},"page":"244","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Antioxidants Reduce Muscular Dystrophy in the dy2J\/dy2J Mouse Model of Laminin \u03b12 Chain-Deficient Muscular Dystrophy"],"prefix":"10.3390","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9255-0517","authenticated-orcid":false,"given":"Vahid","family":"M. Harandi","sequence":"first","affiliation":[{"name":"Unit of Muscle Biology, Department of Experimental Medical Science, Lund University, 221 84 Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1606-432X","authenticated-orcid":false,"given":"Bernardo","family":"Moreira Soares Oliveira","sequence":"additional","affiliation":[{"name":"Unit of Muscle Biology, Department of Experimental Medical Science, Lund University, 221 84 Lund, Sweden"},{"name":"Functional Genomics &amp; Metabolism Unit, Department of Biochemistry &amp; Molecular Biology, University of Southern Denmark, 5230 Odense, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Val\u00e9rie","family":"Allamand","sequence":"additional","affiliation":[{"name":"Unit of Muscle Biology, Department of Experimental Medical Science, Lund University, 221 84 Lund, Sweden"},{"name":"Centre de Recherche en Myologie, Sorbonne Universit\u00e9, Inserm, UMRS974, 75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ariana","family":"Friberg","sequence":"additional","affiliation":[{"name":"Unit of Muscle Biology, Department of Experimental Medical Science, Lund University, 221 84 Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1112-6015","authenticated-orcid":false,"given":"Cibely C.","family":"Fontes-Oliveira","sequence":"additional","affiliation":[{"name":"Unit of Muscle Biology, Department of Experimental Medical Science, Lund University, 221 84 Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Madeleine","family":"Durbeej","sequence":"additional","affiliation":[{"name":"Unit of Muscle Biology, Department of Experimental Medical Science, Lund University, 221 84 Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,3,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1016\/bs.ctm.2015.05.002","article-title":"Laminin-alpha2 Chain-Deficient Congenital Muscular Dystrophy: Pathophysiology and Development of Treatment","volume":"76","author":"Durbeej","year":"2015","journal-title":"Curr. 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