Expression of dystrophin, beta-spectrin, merosin, and alpha- and beta-sarco
glycans 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 m
embranes in various vacuolar myopathies. Caveolin-3 and dystrophin were exp
ressed on the vacuolar membranes in lysosomal glycogen storage disease with
normal acid maltase, hypokalemic myopathy, and centronuclear myopathy. Sar
coglycans and merosin were expressed only on the vacuolar membrane in lysos
omal glycogen storage disease with normal acid maltase. Immunostain of cave
olin-3 and sarcoglycans is therefore useful for differential diagnosis of v
acuolar myopathies. (C) 1999 John Wiley & Sons, Inc.