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Figure 7


Fig. 7. Functional and compositional analyses of MTJs. (A-D) Evans blue analysis reveals normal MTJ integrity in a control mouse diaphragm (A), but evidence of damage is observed in Lamb2-/- (B) and Lamb2-/-; MCK-B2 (C) mice at 3 weeks of age. A diaphragm from an older adult Lamb2-/-; MCK-B2; NEPH-B2 mouse shows no damage (D), suggesting eventual repair of MTJs. (E,F) Double staining for laminin ß2 (green) and laminin {alpha}2 (red) in intercostal muscles reveals concentration of ß2 at MTJs in both control (E) and Lamb2-/-; MCK-B2 (F) mice. (G,H) Double staining for mouse plus rat ß2 (G) and rat ß2 only (H) in a Lamb2+/-; MCK-B2 mouse shows that many MTJs contain little if any transgene-derived protein. (I-L) Double staining for total laminin ß2 (green) and {alpha}2 (red) in intercostal muscles from aged control (I,J) and Lamb2-/-; MCK-B2; NEPH-B2 (K,L) mice reveals efficient accumulation of ß2 at MTJs. Transgene-derived ß2 is missing from extra-junctional BMs in rescued mutant muscle fibers (F,K). Scale bars: in D, 1 mm for A-D; in H, 50 µm for A-I,K; in L, 20 µm for J,L.