Loss of Calpain 3 Proteolytic Activity Leads to Muscular Dystrophy and to Apoptosis-Associated Iκbα/Nuclear Factor κb Pathway Perturbation in Mice

@article{Richard2000LossOC,
  title={Loss of Calpain 3 Proteolytic Activity Leads to Muscular Dystrophy and to Apoptosis-Associated Iκbα/Nuclear Factor κb Pathway Perturbation in Mice},
  author={Isabelle Richard and Carinne Roudaut and Sylvie Marchand and Stephen Baghdiguian and Muriel Herasse and Daniel Stockholm and Yasuko Ono and Laurence Suel and Nathalie Bourg and Hiroyuki Sorimachi and G{\'e}rard Lefranc and Michel Fardeau and Alain S{\'e}bille and Jacques S. Beckmann},
  journal={The Journal of Cell Biology},
  year={2000},
  volume={151},
  pages={1583 - 1590}
}
Calpain 3 is known as the skeletal muscle-specific member of the calpains, a family of intracellular nonlysosomal cysteine proteases. It was previously shown that defects in the human calpain 3 gene are responsible for limb girdle muscular dystrophy type 2A (LGMD2A), an inherited disease affecting predominantly the proximal limb muscles. To better understand the function of calpain 3 and the pathophysiological mechanisms of LGMD2A and also to develop an adequate model for therapy research, we… CONTINUE READING
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