PMC2816849_01
Muscle specimens of the quadriceps femoris muscle (from patient No. 1) stained by hematoxylin-eosin (A) and modified Gomori Trichrome (B). There were great variation in the size of fibers, fiber splitting with internal nuclei, and clumps of small fibers (x200).
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F1
All the patients showed the following non-specific myopathic changes in the muscle: increased internal nuclei, a few or many atrophic fibers, degenerating fibers with regenerating fibers, and an increased fiber size variation (Fig. 1).
Immunostaining against alpha-sarcoglycan (alpha-SG), beta-sarcoglycan (beta-SG), caveolin-3 (Cav-3), and beta-dystroglycan (beta-DG) on the muscle specimen in the four LGMD2B patients by the diaminobenzidine (DAB)-peroxidase method. All four LGMD2B patients showed a normal immunoreactivity against alpha-sarcoglycan, beta-sarcoglycan, caveolin-3, and beta-dystroglycan (x200).
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PMC2816849_01_jkms-19-447-g002.jpg
F2