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Fig. 1. mdx/ ${alpha}$ 7^-/- mice lack dystrophin and ${alpha}$ 7 integrin. (A) Multiplex PCR and ARMS assays were performed on genomic tail DNA to detect wild-type versus ${alpha}$ 7-integrin-targeted allele and the wild-type versus mdx mutation, respectively. The wild-type ${alpha}$ 7-integrin allele (727 bp) was detected in wild-type and mdx mice, whereas the targeted ${alpha}$ 7-integrin allele (482 bp) was detected in the ${alpha}$ 7^-/- and double-knockout mice. Wild-type dystrophin (275 bp) was detected in wild-type and ${alpha}$ 7^-/- mice, whereas the mdx mutation (275 bp) was present in the mdx and double-knockout mice. Neg, negative control lacking DNA. (B) Western blotting with anti- ${alpha}$ 7A-integrin and ${alpha}$ 7B-integrin antibodies in gastrocnemius muscle of 3-week-old mice confirmed the absence of ${alpha}$ 7-integrin protein in ${alpha}$ 7^-/- and mdx/ ${alpha}$ 7^-/- mice. (C) Immunofluorescence with anti-CA5.5 antibody, which recognizes ${alpha}$ 7 integrin, demonstrated loss of the ${alpha}$ 7 integrin in triceps muscle from 3-week-old ${alpha}$ 7^-/- and mdx/ ${alpha}$ 7^-/- mice. The absence of dystrophin in mdx and mdx/ ${alpha}$ 7^-/- mice was verified by immunofluorescence with an anti-dystrophin antibody. Bar, 10 µm.

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