Figure 1.

Loss of cyclophilin D reduces pathology in Scgd^−/− muscle. (a) Mitochondrial swelling, as assessed by light scattering at 540 nm, of mitochondria isolated from muscle of 6-week-old wild-type (WT), Scgd^−/−, and Scgd^−/−Ppif ^−/− mice. (b) Muscle weight/tibia length ratio (MW/TL) measurements for gastrocnemius (Gastroc.) and quadriceps (Quad.) of WT (4 males, 2 females), Scgd^−/− (7 males), Scgd^−/−Ppif ^−/− (6 males) and Ppif ^−/− (1 male, 2 females) mice at 6 weeks of age. (c) MW/TL measurements for gastrocnemius and quadriceps of WT (5 males, 4 females), Scgd^−/− (5 males, 5 females), Scgd^−/−Ppif ^−/− (5 males, 5 females) and Ppif ^−/− (1 male, 2 females) mice at 8 months of age. (d) Percentage of fibers containing central nuclei in the gastrocnemius of 6-week-old WT, Scgd^−/−, and Scgd^−/−Ppif ^−/− mice. (e) Fiber areas (grouped into size ranges) from diaphragms of 6-week-old WT (4 males, 2 females), Scgd^−/− (7 males) and Scgd^−/−Ppif ^−/− (6 males) mice. (f) Fiber numbers in the gastrocnemius muscle of WT, Scgd^−/−, and Scgd^−/−Ppif ^−/− mice. (g) Representative H&E-stained histology of the indicated muscles from the indicated cohorts of mice at 6 weeks of age. Diaph., diaphragm. Scale bars, 100 μm. (h) Specific force of isolated EDL muscles from 6 male mice in each group (11 or 12 muscles analyzed in total per group) to assess muscle strength. *P < 0.05 versus WT; #P < 0.05 versus Scgd^−/−. The key in a applies to all panels. The sample number for each group is indicated inside each bar. Error bars represent s.e.m.