Figure 2. The dystrophic histopathology in mdx^4cv mice was profoundly improved by the absence of desmin.

A) Frozen sections demonstrating the histopathology of skeletal muscles. Note that the extensive central nucleation and mononuclear cell infiltrate, calcification, necrosis and inflammation in mdx^4cv muscles were significantly diminished in the dko skeletal muscles. All panels are representative sections of gastrocnemius muscle except the second row, which are sections of the diaphragm. Scale bars  = 100 µm. B) The number of centrally nucleated fibers was significantly diminished in hind-limb and respiratory muscles in the dko mice when compared with the mdx^4cv muscles C) Calcified fibers were found in the mdx^4cv diaphragm muscle but not in the dko muscles. D) Quantitation of the total number of necrotic fibers in the gastrocnemius muscles. E) Quantitation of macrophages in the gastrocnemius muscles. F) Quantitation of the CD3 positive T-lymphocytes in the gastrocnemius muscles. N = 4 for all experiments. All bars in the graphs represent mean +/− S.D. *P<0.05, **P<0.01 and ***P<0.001 compared to wild-type; ^# P<0.05, ^## P<0.01 and ^### P<0.001 compared to mdx^4cv.