Calpain 3-deficient (CLPN3 -/-)


These mice have a very mild phenotype with minimal histological appearance of the disease in skeletal muscles. These mice show reduced body weight, mild deficits in forelimb strength and motor coordination around 3 months age. Evidence of atrophy, fiber area variability, necrosis and regeneration typical of LGMD2A biopsies are seen in this model. C57BL/6 mice are the wild-type controls for this model.

Related information

DiFranco, M., Kramerova, I., Vergara, J. L., & Spencer, M. J. (2016). Attenuated Ca2+ release in a mouse model of limb girdle muscular dystrophy 2A. Skelet Muscle, 6(1), 11. Publication

Kramerova, I., Ermolova, N., Eskin, A., Hevener, A., Quehenberger, O., Armando, A. M., . . . Spencer, M. J. (2016). Failure to up-regulate transcription of genes necessary for muscle adaptation underlies limb girdle muscular dystrophy 2A (calpainopathy). Hum Mol Genet, 25(11), 2194-2207. Publication