Fougerousse, Françoise
Gonin, Patrick
Durand, Muriel
Richard, Isabelle
Raymackers, Jean-Marc
[UCL]
Defects in human calpain 3 are responsible for limb-girdle muscular dystrophy type 2A, an autosomal-recessive disorder characterized mainly by late-onset proximal muscular atrophy. A corresponding murine model has previously been generated by gene targeting. In this report, muscular activity of calpain 3-deficient (capn3(-/-)) mice was evaluated at different ages. Growth curves showed a progressive global muscular atrophy. Histological examination throughout the lifespan of mice confirmed the dystrophic lesions. Whole animal tests showed only a mild significant impairment of the forelimbs. Studies of the mechanical properties of selected isolated fast- and slow-twitch muscles demonstrated that slow-twitch muscles were significantly weaker in capn3(-/-) mice than in wild-type mice. Three different tests showed that there was no membrane disruption, suggesting a nonmechanical etiology of capn3(-/-) mice dystrophy. These findings are consistent with a mechanism involving signaling systems.
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Bibliographic reference |
Fougerousse, Françoise ; Gonin, Patrick ; Durand, Muriel ; Richard, Isabelle ; Raymackers, Jean-Marc. Force impairment in calpain 3-deficient mice is not correlated with mechanical disruption.. In: Muscle & nerve, Vol. 27, no. 5, p. 616-23 (2003) |
Permanent URL |
http://hdl.handle.net/2078.1/28112 |