Article
Intact satellite cells lead to remarkable protection against Smn gene defect in differentiated skeletal muscle.
The Journal of cell biology - 12 May 2003
Nicole Sophie, Desforges Benedicte, Millet Gaelle, Lesbordes Jeanne, Cifuentes-Diaz Carmen, Vertes Dora, Cao My Linh, De Backer Fabienne, Languille Laetitia, Roblot Natacha, Joshi Vandana, Gillis Jean-Marie, Melki Judith
Abstract excerpt
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...
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