Skeletal Muscle Is a Primary Target of SOD1G93A-Mediated Toxicity (Articolo in rivista)

Type
Label
  • Skeletal Muscle Is a Primary Target of SOD1G93A-Mediated Toxicity (Articolo in rivista) (literal)
Anno
  • 2008-01-01T00:00:00+01:00 (literal)
Alternative label
  • Dobrowolny G. 1, Aucello M. 1, Rizzuto E. 1, Beccafico S. 2, Mammucari C.3, Bonconpagni S.2, Belia S.3, Wannenes F. 4, NicolettiC. 1, Del Prete Z. 5, Rosenthal N.6, Molinaro M. 1, Protasi F.2, Fanò G. 2, Sandri M. 7,8,9, Musarò A.1,10 (2008)
    Skeletal Muscle Is a Primary Target of SOD1G93A-Mediated Toxicity
    (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
  • Dobrowolny G. 1, Aucello M. 1, Rizzuto E. 1, Beccafico S. 2, Mammucari C.3, Bonconpagni S.2, Belia S.3, Wannenes F. 4, NicolettiC. 1, Del Prete Z. 5, Rosenthal N.6, Molinaro M. 1, Protasi F.2, Fanò G. 2, Sandri M. 7,8,9, Musarò A.1,10 (literal)
Pagina inizio
  • 425 (literal)
Pagina fine
  • 436 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume
  • 8(5) (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#note
  • Issued 5 November 2008 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
  • 1 = Institute Pasteur Cenci-Bolognetti, Department of Histology and Medical Embryology, CE-BEMM and IIM, Sapienza University of Rome, Via A. Scarpa, 14 Rome 00161, Italy 2 = Ce.S.I. Centro Scienze dell'Invecchiamento, IIM, Università degli Studi G. d'Annunzio, Chieti 66100, Italy 3 = Laboratorio Interuniversitario di Miologia, Università di Perugia, 06100 Italy 4 = Department of Sciences of Human Movement and Sport, IUSM and INMM-CNR, Rome 00100, Italy 5 = Department of Mechanical Engineering, Sapienza University of Rome, Rome 00184, Italy 6 = EMBL Mouse Biology Program, Monterotondo 00016, Italy 7 = Dulbecco Telethon Institute, University of Padova, Padova 35100, Italy 8 = Venetian Institute of Molecular Medicine, Padova 35100, Italy 9 = Department of Biomedical Science, Padova 35100, Italy 10 = Edith Cowan University, Perth, Western Australia 6027, Australia (literal)
Titolo
  • Skeletal Muscle Is a Primary Target of SOD1G93A-Mediated Toxicity (literal)
Abstract
  • The 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. The analysis of molecular pathways associated with muscle atrophy revealed that accumulation of oxidative stress served as signaling molecules to initiate autophagy, one of the major intracellular degradation mechanisms. These data demonstrate that skeletal muscle is a primary target of SOD1G93A -mediated toxicity and disclose the molecular mechanism whereby oxidative stress triggers muscle atrophy. (literal)
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