Force impairment in calpain 3-deficient mice is not correlated with mechanical disruption.
(2003) Muscle & Nerve — Vol. 27, n° 5, p. 616-623 (2003)
Files
28112.pdf
Restricted Access - Adobe PDF
- 5.41 MB
Details
- Authors
- Abstract
- 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.
- Affiliations
Citations
Fougerousse, F., Gonin, P., Durand, M., Richard, I., & Raymackers, J.-M. (2003). Force impairment in calpain 3-deficient mice is not correlated with mechanical disruption. Muscle & Nerve, 27(5), 616-623. https://doi.org/10.1002/mus.10368 (Original work published 2003)