Skeletal Muscle Is a Primary Target of SOD1G93A-Mediated Toxicity
2009; Cell Press; Volume: 9; Issue: 1 Linguagem: Inglês
10.1016/j.cmet.2008.12.003
ISSN1932-7420
AutoresGabriella Dobrowolny, Michela Aucello, Emanuele Rizzuto, Sara Beccafico, Cristina Mammucari, Simona Boncompagni, Silvia Belia, Francesca Wannenes, Carmine Nicoletti, Zaccaria Del Prete, Nadia Rosenthal, Mario Molinaro, Feliciano Protasi, Giorgio Fanò, Marco Sandri, Antonio Musarò,
Tópico(s)Amyotrophic Lateral Sclerosis Research
Resumo(Cell Metabolism 8, 425–437; November 5, 2008) In the preparation of this manuscript, coauthor Simona Boncompagni's name had been misspelled. In addition, coauthor Cristina Mammucari's correct affiliation is Department of Biomedical Science, Padova 35100, Italy. These corrections do not affect the conclusions in the paper, and we apologize for any confusion the errors might have caused. Skeletal Muscle Is a Primary Target of SOD1G93A-Mediated ToxicityDobrowolny et al.Cell MetabolismNovember 05, 2008In BriefThe antioxidant enzyme superoxide dismutase 1 (SOD1) is a critical player of the antioxidative defense whose activity is altered in several chronic diseases, including amyotrophic lateral sclerosis. However, how oxidative insult affects muscle homeostasis remains unclear. This study addresses the role of oxidative stress on muscle homeostasis and function by the generation of a transgenic mouse model expressing a mutant SOD1 gene (SOD1G93A) selectively in skeletal muscle. Transgenic mice developed progressive muscle atrophy, associated with a significant reduction in muscle strength, alterations in the contractile apparatus, and mitochondrial dysfunction. Full-Text PDF Open Archive
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