Titre :
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Blocking the myostatin signal permits to improve the success of myoblast transplantation in mdx (abstract : congrès international de Myologie, 2005)
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contenu dans :
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Auteurs :
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Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ;
Benabdallah BF ;
Bouchentouf M ;
Tremblay J
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Type de document :
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Article
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Année de publication :
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2005
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Pages :
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p. 111
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Langues:
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Anglais
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Mots-clés :
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colloque
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dystrophine
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muscle squelettique
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myoblaste
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régénération musculaire
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souris mdx
;
thérapie cellulaire
;
traumatisme musculaire
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Résumé :
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Communication n° 170. Background : Duchenne muscular dystrophy is caused by a dystrophin gene mutation. Transplantation of normal myoblasts results in long-term restoration of dystrophin. However, the success of this approach is compromised by the limited time of regeneration following muscle damage. Myostatin is known to be responsible for this limited skeletal muscle regeneration. Our purpose is to verify whether blocking the myostatin signal in mdx host mice or in normal myoblasts transplanted in mdx host mice would increase the extent of muscle repair and thus permits to form more dystrophin positive fibers. Methods : Transgenic mdx mice carrying a dominant negative form of myostatin receptor (dnActRIIB) were used to test the fiber resistance to damage and as a host for normal myoblast transplantation. Myoblasts obtained from non dystrophic transgenic mice carrying the dominant negative myostatin receptor were also transplanted in non transgenic mdx mice. Results : Transgenic mdx mice carrying the dnActRIIB gene have bigger muscles than mdx mice with the normal gene of ActRIIB. Their fiber resistance to exercise induced damage was also greatly improved. Moreover, the success of normal myoblast transplantation was significantly enhanced in mdx/dnActRIIB mice. Finally, non-dystrophic dnActRIIB myoblasts formed more abundant and bigger dystrophin positive fibers when transplanted in mdx mice. Conclusions : Blocking the myostatin signal in mdx mice permitted to increase the size of muscle fibers and the fiber resistance to damage induced by exercise, and improved the success of normal myoblast transplantation. The transplantation in mdx mice of dnActRIIB myoblasts formed more abundant and larger dystrophin positive fibers.
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