Figure 3.

Genetic ablation of cyclophilin D increases lifespan and reduces muscular dystrophy pathology in Lama2^−/− mice. (a) Mitochondrial swelling, as assessed by light scattering at 540 nm, of mitochondria isolated from skeletal muscle of the indicated groups of mice. The key for all panels is shown in the lower right corner of the figure. (b) Survival rate for Lama2^−/−, Lama2^−/−Ppif ^+/− and Lama2^−/−Ppif ^−/− mice. P o 0.001 for Lama2^−/− versus Lama2^−/−Ppif ^−/−. (c) Body weights of Lama2^−/−, Lama2^−/−Ppif ^+/− and Lama2^−/−Ppif ^−/− mice at 4 weeks of age. WT mice at the same age had an average body weight of 14 g. (d) MW/TL for gastrocnemiussoleus (Gas.-sol.), quadriceps (Quad.) and tibialis anterior (TA) from WT (6 males, 4 females), Lama2^−/− (5 males, 2 female) and Lama2^−/−Ppif ^−/− (4 male, 2 female) mice at 3 weeks of age. (e) Fiber numbers in the gastrocnemius muscle of WT (6 male), Lama2^−/− (4 male) and Lama2^−/−Ppif ^−/− (4 male) mice at 3 weeks of age. (f) Fiber areas of gastrocnemius (grouped into 2 size ranges) from 3-week-old WT, Lama2^−/− and Lama2^−/−Ppif ^−/− mice. (g) Hydroxyproline content from diaphragm and gastrocnemius of 3-week-old WT, Lama2^−/− and Lama2^−/−Ppif ^−/− mice. (h) Myeloperoxidase (MPO) activity from quadriceps of 3-week-old WT, Lama2^−/− and Lama2^−/−Ppif ^−/− mice. *P o 0.05 versus WT; #P o 0.05 versus Lama2^−/−. The sample number for each group is indicated inside each bar. Error bars represents s.e.m.