Fig. 8.

Myocytes of CPVT mice have increased mito-ROS emission rates and RyR2 oxidation in comparison to controls. a Representative recording of ROS production measured with MitoSOX in ISO-treated (100 nmol/L) WT and CPVT VMs. Signal was normalized to maximum fluorescence obtained on application of DTDP (200 µmol/L). The ROS production rate (%) was calculated by normalization to the maximum rate of ROS emission observed on application of DTDP, and rates for these representative recordings are indicated. The graph in b depicts mean data ± SEM for the normalized ROS production rate of WT and CPVT VMs. N = 3 WT, 5 CPVT animals, n = 7–13 VMs. *p < 0.05 vs. WT group, paired Student’s t test. c Representative images of immunoprecipitated RyR2 from freshly isolated ISO-treated WT and CPVT VMs, immunoblotted for RyR2 signal and oxidation using DNP antibody. The graph in d depicts quantification of normalized DNP signal (%). N = 6 animals per group. *p < 0.05 vs. WT group, two sample Student’s t test. e Representative western blots from WT and CPVT VMs probed for MCUb and MCU expression. The graph in f depicts quantification of normalized MCUb/MCU signal (%). N = 5 animals per group. *p < 0.05 vs. Ctrl group, two-sample Student’s t test