Figure 4. Electrically evoked peak Ca²⁺ transients in muscle fibers from treated double heterozygous (dHT) compound heterozygous (dHT) mice was rescued by TMP269 + 5-aza-2-deoxycytidine (5-Aza) administration.

Enzymatically dissociated flexor digitorum brevis (FDB) fibers dissected from 4 to 6 mice per group, were loaded with Mag-Fluo-4 and electrically stimulated by field stimulation. Black line, vehicle-treated wild type (WT), gray line, vehicle-treated dHT, red line, TMP269 + 5-Aza-treated dHT. (A) Representative Ca²⁺ transient evoked by a single pulse (twitch) of 50 V with a duration of 1 ms. (B) Whisker plots of peak twitch. Each symbol represents results obtained from a single FDB fiber. (C) Representative Ca²⁺ transient evoked by tetanic stimulation by a train of pulses delivered at 100 Hz for 300 ms. (D) Whisker plots of peak transient induced by tetanic stimulation. Each symbol represents results obtained from a single FDB fiber. *p < 0.05 (ANOVA followed by the Bonferroni post hoc test). The exact p values are given in Supplementary file 4.