Tomé et al. (2009) reported that MSH2 ATPase domain mutation could influence somatic instability in DM1 transgenic mice. Compared with fibroblasts, MSH2, MSH3, and MSH6, were highly expressed in DM1 patient-derived iPSC, accompanied with longer CTG repeat, while MSH2 silencing inhibited CTG repeat expansion (Du et al., 2013). Flower et al. (2019) reported that the altered MSH3 levels caused by MSH3 3a repeat allele decreased somatic expansion and changed the DM1 phenotype. This evidence concerns the gene MSH2 and myotonic dystrophy type 1.