DMPK and myotonic dystrophy type 1: In homozygous DMSXL mice, the ubiquitous expression of expanded DMPK transcripts from the human DM1 locus results in multisystemic phenotypes reproducing molecular alterations as well as many aspects of the human pathology including reduced muscle strength, lower motor performances, peripheral neuropathy, cardiac conduction defects, cognitive and behavioural abnormalities.32, 37, 38, 39, 40, 41