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Fig. 2. Muscle-wasting and reduced viability in mdx/ ${alpha}$ 7^-/- mice. (A) Photograph of mdx and mdx/ ${alpha}$ 7^-/- littermates aged 18 days. Double-knockout mice appear smaller than mdx littermates and show outward signs of kyphosis. These mice also exhibit joint contractures, reduced mobility and tremors (see supplementary material, Movie 1). (B) The average weight of 10-day-old male mdx/ ${alpha}$ 7^-/- mice was indistinguishable from that of mdx littermates. The weight of both mdx and mdx/ ${alpha}$ 7^-/- mice was significantly different from wild-type and ${alpha}$ 7^-/- animals (^*P<0.05). (C) The average weight of 21-day-old male mdx/ ${alpha}$ 7^-/- mice was significantly different from wild-type, mdx, and ${alpha}$ 7^-/- animals (^*P<0.05), indicating that double-knockout mice failed to gain weight. (D) The viability of mdx/ ${alpha}$ 7^-/- mice (n=21) is severely reduced compared with wild-type (n=12), ${alpha}$ 7^-/- (n=148) and mdx (n=19) mice. All mdx/ ${alpha}$ 7^-/- mice died at 16-26 days.

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