Fig. 3

Histopathological examination of the skeletal muscles. a: HE staining showing muscle fibers of variable sizes, abnormal material deposits (white arrow), and angular atrophic fibers (red arrow). b: MGT staining showing purple myofibrillary inclusions. c: NADH-TR staining showing reduced oxidative enzyme activities in some fibers, visible as core-like lesions (red arrow). d: Immunohistochemistry staining with an anti-FLNC antibody revealing immunoreactive deposits at both subsarcolemmal and sarcoplasmic levels. e: Ultrastructural analysis of a muscle fiber showing myofibrillar disorganization (arrows) and submuscular lipofuscin deposition (star). Magnification, × 5000