| First Author | Fougerousse F | Year | 2003 |
| Journal | Muscle Nerve | Volume | 27 |
| Issue | 5 | Pages | 616-23 |
| PubMed ID | 12707983 | Mgi Jnum | J:105529 |
| Mgi Id | MGI:3615747 | Doi | 10.1002/mus.10368 |
| Citation | Fougerousse F, et al. (2003) Force impairment in calpain 3-deficient mice is not correlated with mechanical disruption. Muscle Nerve 27(5):616-23 |
| abstractText | 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. |
