DMPK and Myotonia: Furthermore, the systemic treatment with ASO (ISIS 486178) targeted to the non-CUG sequence within the 3′-UTR of DMPK also specifically rescued DM1 phenotypes of myotonia and cardiac conduction defects in DM200 mice [a DM1 model carrying a GFP-DMPK 3′-UTR (CTG) 200 transgene] (Jauvin et al., 2017; Yadava et al., 2020).