Disrupted expression of dysferlin and dystrophin in skeletal muscle of dystrophin/dysferlin double-knockout mice. (A) Western blot showing the expression pattern of dysferlin (DysF), dystrophin (Dyst), β-dystroglycan (β-DG) and dihydropyridine receptor α2 (DHPRα2) in skeletal muscle tissue lysates from wild-type (WT), dysferlin-null, mdx and dystrophin/dysferlin double-knockout (DKO) mice. (B) Expression of dystrophin and dysferlin in skeletal muscles from WT, dysferlin-null, mdx and DKO mice were examined by immunofluorescence staining. (C) β-DG and sarcospan (SSPN) were greatly diminished at the sarcolemma of mdx and DKO muscles, but laminin α2 (Lam2) staining was not reduced. Scale bars: 100 μm.