Cyclosporine A treatment improves survival of the SMNKO mice.A) Survival curve of WT and SMNKO mice given drinking water supplemented with or without 210 mg/L Cyclosporine A (CsA) (n = 30–31). B) ATP levels in quadriceps muscle from WT and SMNKO mice treated +/− CsA (6 or 12 weeks of age, n = 6–13). C) AMPK pT172 levels in quadriceps muscle from WT and SMNKO mice treated +/− CsA (6 or 12 weeks of age, n = 6–13). D) Average fiber area in quadriceps muscle of WT and SMNKO mice treated +/− CsA in the drinking water (6 or 12 weeks of age, n = 5–7). E) Quantification of central nuclei of quadriceps muscle of WT and SMNKO mice given +/− CsA in the drinking water (6 or 12 weeks of age, n = 5–7). F) Protein levels of Cyclophilin D in gastrocnemius muscle from WT and SMNKO mice given +/− CsA in the drinking water (6 or 12 weeks of age, n = 6–13). G) Quantification of IgG+ positive cells in quadriceps from WT and SMNKO mice treated +/− CsA (6 or 12 weeks of age, n = 5–7). H) Representative images from IgG staining of Quadriceps muscle of WT and SMNKO mice treated +/− CsA. Error bars represent SEM. ∗ Difference to WT control mice. # Main effect of genotype. $Main effect of CsA.