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Figure 4

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Cryostat sections of skeletal and cardiac muscle from patient 2 (PT) and control (CNTR), immunolabeled with antibodies against LAMP-2 and caveolin-3 (CAV-3). LAMP-2 reaction was absent in muscle and heart from the patient compared with the diffuse positive reaction observed in control. Caveolin-3 immunolabeling was positive in the plasma membrane of muscle fibers and cardiomyocytes of both patient and control. In the skeletal muscle from Danon disease, caveolin-3 labeling was observed also in the membrane of the lysosomes that delineate most vacuoles. The cellular membranes that make up both the plasmalemma and the lysosomes are often in continuity. Microscope magnification, ×400.

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