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. 2021 Jan 4;29(24):3919–3934. doi: 10.1093/hmg/ddaa278

Figure 6.

Figure 6

Effect of S107 on RyR1 remodeling and isometric contractile properties of diaphragm from female LmnaH222P/H222P mice. (A) Immunoblot showing RyR1 immunoprecipitated from extracts of diaphragms of female WT mice and LmnaH222P/H222P mice treated with either placebo (LmnaH222P) or S107 (LmnaH222P + S107) and oxidation (DNP), PKA-catalyzed phosphorylation (pSer2844) and calstabin1 depletion. Samples are from mice at 30 weeks of age; treated mice received placebo or drug for 8 weeks. Migration of molecular mass standards are indicated to the left of the blot. (B–D) Quantification of associated calstabin1 (B), oxidized (C) and phosphorylated (D) RyR1 normalized to total RyR1 from WT mice and LmnaH222P/H222P mice treated with placebo (LmnaH222P) or S107 (LmnaH222P + S107). Values are means ± SEM (Control n = 3, LmnaH222P n = 3, LmnaH222P + S107 n = 3); *P < 0.05; ns not significant. (E–G) Representative records of diaphragmatic specific force production measured ex-vivo at 20 and 120 Hz in muscle bundles under isometric conditions of WT (E), LmnaH222P/H222P mice treated with placebo (LmnaH222P) (F) or S107 (LmnaH222P + S107) (G). (H) Average force-frequency relationship recorded in WT, LmnaH222P/H222P mice treated with placebo (LmnaH222P) or S107 (LmnaH222P + S107). Values are means ± SEM (Control n = 5, LmnaH222P n = 7, LmnaH222P + S107 n = 7); *P < 0.05, LmnaH222P versus LmnaH222P + S107.