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The treatment with S107 was efficient only when administered earlier in very young animals (from 2 to 6months of age) and prevented the segmental alterations seen in non-treated mdx mice. This is the first animal study to evaluate the therapeutic effect of a drug targeting early onset of DMD-related cardiomyopathy, using 2D strain echocardiography. Speckle-tracking analyses revealed early alterations of LV posterior segments that could be prevented by 4months of RyR2 stabilization. This is the first animal study to evaluate the therape