Neural stem cells (NSC) derived from Embryonic Stem cells (ES) or from induced Pluripotent Stem Cells (iPSCs) developed in the context of DM1, have shown to recapitulate important features of DM1 (RNA foci, MBNL sequestration, splicing defect; Marteyn et al., 2011; Denis et al., 2013; Xia et al., 2013; Xia and Ashizawa, 2015). This evidence concerns the gene MBNL1 and myotonic dystrophy type 1.