{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T14:18:08Z","timestamp":1791296288724,"version":"4.1.0"},"reference-count":33,"publisher":"Rockefeller University Press","issue":"3","content-domain":{"domain":["rupress.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2003,5,12]]},"abstract":"<jats:p>Deletion of murine Smn exon 7, the most frequent mutation found in spinal muscular atrophy, has been directed to either both satellite cells, the muscle progenitor cells and fused myotubes, or fused myotubes only. When satellite cells were mutated, mutant mice develop severe myopathic process, progressive motor paralysis, and early death at 1 mo of age (severe mutant). Impaired muscle regeneration of severe mutants correlated with defect of myogenic precursor cells both in vitro and in vivo. In contrast, when satellite cells remained intact, mutant mice develop similar myopathic process but exhibit mild phenotype with median survival of 8 mo and motor performance similar to that of controls (mild mutant). High proportion of regenerating myofibers expressing SMN was observed in mild mutants compensating for progressive loss of mature myofibers within the first 6 mo of age. Then, in spite of normal contractile properties of myofibers, mild mutants develop reduction of muscle force and mass. Progressive decline of muscle regeneration process was no more able to counterbalance muscle degeneration leading to dramatic loss of myofibers. These data indicate that intact satellite cells remarkably improve the survival and motor performance of mutant mice suffering from chronic myopathy, and suggest a limited potential of satellite cells to regenerate skeletal muscle.<\/jats:p>","DOI":"10.1083\/jcb.200210117","type":"journal-article","created":{"date-parts":[[2003,5,12]],"date-time":"2003-05-12T15:38:10Z","timestamp":1052753890000},"page":"571-582","update-policy":"https:\/\/doi.org\/10.1083\/jcb.crossmarkpolicy","source":"Crossref","is-referenced-by-count":99,"title":["Intact satellite cells lead to remarkable protection against\n                    <i>Smn<\/i>\n                    gene defect in differentiated skeletal muscle"],"prefix":"10.1083","volume":"161","author":[{"given":"Sophie","family":"Nicole","sequence":"first","affiliation":[{"name":"1Laboratoire de Neuroge\u0301ne\u0301tique Mole\u0301culaire, Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale (INSERM), Universite\u0301 d'Evry, E0223, GENOPOLE, 91057 Evry, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benedicte","family":"Desforges","sequence":"additional","affiliation":[{"name":"1Laboratoire de Neuroge\u0301ne\u0301tique Mole\u0301culaire, Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale (INSERM), Universite\u0301 d'Evry, E0223, GENOPOLE, 91057 Evry, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gaelle","family":"Millet","sequence":"additional","affiliation":[{"name":"1Laboratoire de Neuroge\u0301ne\u0301tique Mole\u0301culaire, Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale (INSERM), Universite\u0301 d'Evry, E0223, GENOPOLE, 91057 Evry, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeanne","family":"Lesbordes","sequence":"additional","affiliation":[{"name":"2Cochin Institute, Departement of Genetics, Development and Molecular Pathology, INSERM U567, CNRS, UMR 8104, University of Paris V, 75014 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmen","family":"Cifuentes-Diaz","sequence":"additional","affiliation":[{"name":"1Laboratoire de Neuroge\u0301ne\u0301tique Mole\u0301culaire, Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale (INSERM), Universite\u0301 d'Evry, E0223, GENOPOLE, 91057 Evry, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dora","family":"Vertes","sequence":"additional","affiliation":[{"name":"3Department of Physiology, Catholic University of Louvain, 1200 Brussels, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"My Linh","family":"Cao","sequence":"additional","affiliation":[{"name":"3Department of Physiology, Catholic University of Louvain, 1200 Brussels, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabienne","family":"De Backer","sequence":"additional","affiliation":[{"name":"3Department of Physiology, Catholic University of Louvain, 1200 Brussels, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laetitia","family":"Languille","sequence":"additional","affiliation":[{"name":"1Laboratoire de Neuroge\u0301ne\u0301tique Mole\u0301culaire, Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale (INSERM), Universite\u0301 d'Evry, E0223, GENOPOLE, 91057 Evry, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natacha","family":"Roblot","sequence":"additional","affiliation":[{"name":"1Laboratoire de Neuroge\u0301ne\u0301tique Mole\u0301culaire, Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale (INSERM), Universite\u0301 d'Evry, E0223, GENOPOLE, 91057 Evry, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vandana","family":"Joshi","sequence":"additional","affiliation":[{"name":"1Laboratoire de Neuroge\u0301ne\u0301tique Mole\u0301culaire, Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale (INSERM), Universite\u0301 d'Evry, E0223, GENOPOLE, 91057 Evry, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Marie","family":"Gillis","sequence":"additional","affiliation":[{"name":"3Department of Physiology, Catholic University of Louvain, 1200 Brussels, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judith","family":"Melki","sequence":"additional","affiliation":[{"name":"1Laboratoire de Neuroge\u0301ne\u0301tique Mole\u0301culaire, Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale (INSERM), Universite\u0301 d'Evry, E0223, GENOPOLE, 91057 Evry, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"291","published-online":{"date-parts":[[2003,5,12]]},"reference":[{"key":"2023072214115750400_BIB1","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1083\/jcb.200202092","volume":"159","year":"2002","journal-title":"J. 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