Figure 2.

Analysis of skeletal membrane fragility, ultrastructural defects and cardiac defects. (a) Electron microscopy was performed on gastrocnemius muscle from 6-week-old WT, Scgd^−/−, and Scgd^−/−Ppif ^−/− mice. The arrowheads show individual mitochondria. Scale bars, 2 μm. (b) Quantification of necrotic myocyte nuclei by hairpin probe hybridization in gastrocnemius histological sections from WT, Scgd^−/− and Scgd^−/−Ppif ^−/− mice. (c) Representative histological fluorescent analysis of myofiber membranes (green) and Evan’s blue dye (red) uptake in the gastrocnemius of 6-week-old Scgd^−/− and Scgd^−/−Ppif ^−/− mice. Scale bars, 100 μm. (d) Quantification of Evan’s blue dye uptake in gastrocnemius and quadriceps muscle from 3 mice in each group after voluntary exercise and expressed as a percentage of fibers. (e) Representative Masson’s trichrome–stained histological sections of hearts from 8-month-old WT, Scgd^−/− and Scgd^−/−Ppif ^−/− mice. Scale bars, 100 μm. (f) Measurement of hydroxyproline content in hearts from the three genotypes analyzed in a expressed as μg hydroxyproline/mg of tissue. Key is shown in b. (g) Echocardiography-measured fractional shortening (FS) in the groups of mice indicated in the key in panel b. *P < 0.05 versus WT; #P < 0.05 versus Scgd^−/−. The sample number for each group is indicated inside each bar. Error bars represent s.e.m.