Both Dmpk-(CTG)480/WT KI and Dmpk-(CTG)480/480 KI reproduce characteristic DM1 pathological molecular signatures, including MBNL sequestration on Dmpk 3’UTR (CUG)480 RNAs, RNA mis-splicing in the vulnerable cell types, and DMPK protein loss65. Here, DMPK is linked to myotonic dystrophy type 1.