CNBP and myotonic dystrophy type 2: In myotonic dystrophy type 2 (DM2), up to 75–11,000 repeat expansions of (TG)n(TCTG)n(CCTG)n reside in intron 1 of ZNF9 (Day and Ranum, 2005), dysregulating alternative slicing as well as protein production by sequestration of the 20S proteasome (Salisbury et al., 2009).