Pathological RNA foci and MBNL sequestration are also observed in the type 2 myotonic dystrophy (DM2), which is caused by the expansion of the CCTG repeats in intron 1 of the CNBP/ZNF9 gene (Liquori et al. 2001; Mankodi et al. 2001; Sznajder and Swanson 2019). Here, CNBP is linked to Proximal myotonic myopathy.