Although DM1 modeling is complicated by the multifaceted impact of the DM1 mutation, many DM1 mouse models have been generated over time through the silencing of the Dmpk gene or the Mbnl family genes; alternatively, mouse models expressing CELF proteins or toxic CTG repeats in various tissues were produced, in order to mimic the different aspects of the disease and to discover therapeutic molecules (Table 1). The gene discussed is DMPK; the disease is myotonic dystrophy type 1.