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Fig. 7 | Acta Neuropathologica Communications

Fig. 7

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

Fig. 7

Autophagic flux studies in human muscle biopsies. a. Western blot for BECN1, LC3I/II and SQSTM1/p62 in Japanese GSDIII patients (P12, P13, P14, P15, P16, P17, P18, P19, P20, P21, P22) and controls. b. Quantification of SQSTM1/p62 decrease and beclin-1 increase levels in GSDIII patients versus controls. Accordingly, an increase in the lipidated form of LC3; LC3II was measured in patients compared to control. c. Western blot for beclin-1, total LC3 and SQSTM1/p62 in five French GSD3 patients (P1, P5, P7, P8 and P 11) and controls

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Acta Neuropathologica Communications

ISSN: 2051-5960

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