Figure 2. The mechanical properties of double heterozygous (dHT) treated with TMP269 + 5-aza-2-deoxycytidine (5-Aza) improve after 15 weeks of treatment.

Mechanical properties of extensor digitorum longus (EDL) and soleus muscle from wild type (WT) and dHT mice treated with vehicle (WT, n = 8; dHT, n = 10) and dHT treated with TMP269 + 5-Aza, n = 13. (A) Representative traces of twitch and (B) maximal tetanic force in EDL (150 Hz) muscle from WT and dHT. Force is expressed as specific force, mN/mm². (C) Statistical analysis of force generated after twitch and (D) tetanic stimulation of isolated EDL muscle. Data points are expressed as Whisker plots (n = 8–13 mice). Each symbol represents the value from a muscle from a single mouse. (E) Representative traces of twitch and (F) maximal tetanic force (120 Hz) of soleus muscle from WT and dHT mice. (G) Whisker plots of force generated after twitch and (H) tetanic stimulation of isolated soleus muscles. Each symbol represents the value from a muscle from a single mouse (n = 8–13 mice). *p < 0.05; **p < 0.01 (ANOVA followed by the Bonferroni post hoc test). The exact p values are given in Supplementary file 2.