PMC9677140_01
A. Frozen section stained with hematoxylin and eosin (H&E) demonstrates prominent size variation of polygonal myofibers and a few basophilic, regenerating fibers. B-C. Dysferlin expression is lost (C) with adequate control (B) by immunofluorescence. D-F. The majority of myofibers express embryonic myosin heavy chain, a marker of regeneration (D), MHC class I sarcolemmal expression is present (E), and C5b-9 deposition on muscle fiber surfaces is identified (F). G. Western blot shows loss of dysferlin (Hamlet monoclonal antibody) with adequate controls, while calpain-3 (full-length 94kd and degradation products) appears Normal (anti-calpain-3 mouse monoclonal antibody 12A2 from Leica Biosystems). The myosin band observed in the immunoblot and the Ponceau S-stained membrane is used to compare the amounts of protein loaded in the lanes of the gel.
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PMC9677140_01_10.1177_2329048X221140298-fig1.jpg
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The University of Iowa; mouse monoclonal F1.652) was diffusely expressed, MHC class I expression was patchy (HLA-ABC; Agilent (DAKO); clone W6/32), and C5b-9 deposits (Abcam; clone aE11; ab66768) were identified on muscle fiber surfaces by immunofluorescence. Taken together, the neuropathologic findings were consistent with a necrotizing myopathy in the context of a dysferlinopathy (Figure 1).