More than 40 mis-splicing events have been confirmed in affected skeletal muscles of DM1 patients (Nakamori et al., 2013); in particular, altered splicing of CLCN1, INSR, BIN1 and DMD pre-mRNAs (Charlet-B et al., 2002; Savkur et al., 2001; Fugier et al., 2011; Rau et al., 2015) have been associated with myotonia, insulin resistance, muscle weakness and muscle fiber disorganization, respectively, which are all typical symptoms of myotonic dystrophy. This evidence concerns the gene DMD and myotonic dystrophy type 1.