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Fig. 3 | Acta Neuropathologica Communications

Fig. 3

From: Deep morphological analysis of muscle biopsies from type III glycogenesis (GSDIII), debranching enzyme deficiency, revealed stereotyped vacuolar myopathy and autophagy impairment

Fig. 3

Ultrastructural findings of GSDIII. a. Cross section of a vacuolated muscle fibers showing myofibrils dissociated by variable sized pools of clear areas evenly distributed in the cytoplasm and subsarcolemmal areas. b. Longitudinal section showing large areas composed of granular material contiguous to the sarcomeric structure. The sarcomeres present a variable width and are sometimes dissociated by accumulated glycogen. c. Cross section showing the presence of dissociated myofibrils due to accumulated glycogen. Few rounded sac-like structures filled with glycogen are also observed

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Acta Neuropathologica Communications

ISSN: 2051-5960

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