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Fig. 3. Defects in the surface organization of basement membrane, receptor and cytoskeletal components in dy^2J muscle. Confocal images of wild-type (wt) and dystrophic (dy2J) muscle fibers. Muscle fibers immunostained to detect laminin- ${alpha}$ 2 (Lm) and corresponding ${alpha}$ -actinin ( ${alpha}$ a), dystrophin (Dys) and corresponding dystroglycan (DG), integrin ß1D, vinculin (Vn), nidogen (Nd), type IV collagen (Col4) and perlecan (Perl). Nonimmune (wild-type) controls for integrin (C1), vinculin (C2), dystrophin/basement membrane components (C3) and dystroglycan (C4) included. Note the extensive but incomplete effacement of rectilinear distributions for surface-distributed basement membrane, receptor and cytoskeletal components with preservation of the sarcomeric ${alpha}$ -actinin cross-band architecture. Arrows in top row panel indicate locations of peripheral nuclei which overlie the sarcomeres. Bar, 2 µm.

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