Figure 2. Characterization of EDM1 mice.

A. Myotonia was measured and scored on a 3 point scale with 0 being no myotonia and 3 being strong, persistent, and prevalent myotonia. EDM1 mice show myotonia as early as 2 weeks of age and reached maximal severity by 4 weeks. B. EDM1 mice have a reduction in grip strength as early as 4 weeks of age while the adult-onset mice did not show a significant reduction. C. PR interval was measured in the EDM1 mice. No statistical difference was measured at 2 weeks of age; however the subsequent time points showed a significant lengthening of the interval. Similar but less severe results were observed in the adult-onset DM1 model. Number of samples (n) provided on the graph, averages listed in the tables below the graphs, error bars are SEM. Two Sample T-Test was used to determine statistical significance with p-values of 0.05 (*), 0.01 (**) or <0.001 (***) displayed.