The LGMD2A dystrophy is characterised by (i) an increase of caspase 3 activity, (ii) a deregulation of the I{kappa}B{alpha}/NF-{kappa}B pathway leading to apoptosis [5, 16], (iii) an increase of membrane permeability, (iv) a decrease in the size of the SR and v) a dysfunction of RyR signalling. This evidence concerns the gene RYR1 and autosomal recessive limb-girdle muscular dystrophy type 2A.