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Caveolin-3 and sarcoglycans in the vacuolar myopathies and centronuclear myopathy
M Inose1, I Higuchi, M Nakagawa
1Third Department of Internal Medicine, Faculty of Medicine, Kagoshima University, Sakuragaoka 8-35-1, Kagoshima 890-8528, Japan.
Abstract:
Expression of dystrophin, beta-spectrin, merosin, and alpha- and beta-sarcoglycans on the vacuolar membranes in some types of vacuolar myopathies has previously been reported. We studied expression of caveolin-3; alpha-, beta-, gamma-, and delta-sarcoglycans; dystrophin; and merosin on the vacuolar membranes in various vacuolar myopathies. Caveolin-3 and dystrophin were expressed on the vacuolar membranes in lysosomal glycogen storage disease with normal acid maltase, hypokalemic myopathy, and centronuclear myopathy. Sarcoglycans and merosin were expressed only on the vacuolar membrane in lysosomal glycogen storage disease with normal acid maltase. Immunostain of caveolin-3 and sarcoglycans is therefore useful for differential diagnosis of vacuolar myopathies.