In particular, in Myotonic Dystrophies triplet expansion within non-coding region of RNAs alters either the activity or the expression of the splicing regulators MBNL1 and CUGBP132 and genome-wide analysis of muscle biopsies from Myotonic Dystrophy type 2-affected patients revealed hundreds of aberrant alternative splicing events.34 Notably, miR-222 expression levels were found increased in several muscular dystrophies.8 Here, we show that targeting Rbm24 by miR-222 is a crucial event in inhibition of muscle-specific transcript production. Here, RBM24 is linked to Proximal myotonic myopathy.