[7]–[10] Omigapil, an inhibitor of the GAPDH-Siah1-mediated apoptosis, was found to be effective in the dyW mouse model. [10] This study also demonstrated significant improvement in functional and histological measures in the dy2J model after therapy with the omigapil (0.1 mg/kg), providing further support for clinical trials of omigapil in congenital muscular dystrophy. This evidence concerns the gene SIAH1 and congenital muscular dystrophy due to LMNA mutation.