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. 2020 Sep 15;11:1014. doi: 10.3389/fneur.2020.01014

Figure 2.

Figure 2

(A) HE staining shows the numerous intramuscular eosinophilic deposits in BAG3opathy (arrow, patient 9); (B–D) immunohistochemistry of serial sections from patient 9 shows aggregation of MFM-related proteins [(B), desmin; (C), BAG3; (D), αB crystallin; different field from (A)], eosinophilic aggregates were most strongly immunoreactive with BAG3 in this patient. Note the aggregates concentrated at the upper myofascicle, while the lower right fascicle appeared normal. (E) HE staining shows fiber size variation and rare regeneration in filaminopathy (patient 11). (F) HE demonstrates eosinophilic materials in FHL1opathy (arrow, patient 13). Note the overall small fiber size. (G) HE shows vacuolated fibers (arrow), central nucleated fibers, and nuclear clumps in HMERF (patient 15). (H) Gomori shows fibers with necklace cytoplasmic bodies (arrow, patient 14). (I,J) HE and NADH demonstrates increased central nuclei and selective type 1 atrophy in titinopathy (patient 16). (K) HE shows rimmed vacuoles (arrow) and esosinophilic bodies (arrowhead) in titinopathy (patient 17). (L) NADH shows relative preservation of the myofibrillar network in the majority of fibers except for occasional bar-like enzyme aggregation (arrow) in patient 17. Magnification: ×200.