RNA-dependent pathophysiological mechanisms and RAN translation of tetrapeptides (polyLPAC and polyQAGR) also coexist in myotonic dystrophy type 2 (DM2, MIM #602668), caused by CCTG/CAGG repeat expansions in CNBP (CCHC-type zinc finger nucleic acid-binding protein, MIM #116955). This evidence concerns the gene CNBP and Proximal myotonic myopathy.