In this regard, DMPK knock-out mice fail to reproduce the multisystem phenotype of DM1 patients, and only develop late-onset myopathy (Jansen et al., 1996; Reddy et al., 1996; Berul et al., 1999), suggesting that haploinsufficiency of DMPK is not the primary mechanism that initiates this disease. Here, DMPK is linked to myotonic dystrophy type 1.