Several RNA-binding proteins including the muscleblind-like protein 1 (MBNL1) and the CUG-triplet repeat RNA-binding protein 1 (CUGBP1) have been recognized as important players in regulating DM1-specific aberrant splicing, consistent with their ability to interact with CUG-repeats (21–32). Here, CELF1 is linked to myotonic dystrophy type 1.