deLonimier et al. have shown that Ψ modification of the expanded intronic CCUG repeat in CNBP, expression of which is associated with type 2 myotonic dystrophy (DM2) in humans, results in reduced binding of muscleblind-like 1 protein to CCUG repeat-expanded CNBP RNA linked to DM2 pathogenicity [174]. The gene discussed is CNBP; the disease is myotonic dystrophy type 2.