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Fig. 4. Severe muscle pathology in mdx/ ${alpha}$ 7^-/- mice. (A) Hematoxylin and eosin staining of tibialis anterior muscle from 3-week-old mice shows the characteristically uniform fiber diameters in wild-type mice. Small necrotic regions and variations in myofiber size were observed in muscle of 3-week-old mdx mice. At this age, muscle from ${alpha}$ 7^-/- mice appeared to be similar to wild-type. By contrast, muscle from mdx/ ${alpha}$ 7^-/- mice exhibited extreme variations myofiber size and large areas of mononuclear cell infiltration (arrow). Bar, 10 µm. (B) Inflammation in the muscle of mdx/ ${alpha}$ 7^-/- mice was quantified by counting the number of CD4-positive immune cells per field of view in triceps muscle of 3-week-old mice. Muscle of wild-type, mdx and ${alpha}$ 7^-/- animals showed less than 1% CD4-positive cells. By contrast, an average of 43.88 CD4-positive immune cells per field was observed in the triceps muscle of mdx/ ${alpha}$ 7^-/- mice, which was significantly higher when compared with control genotypes (^*P<0.05).

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