“…Early changes are central myofibrillar lesions and Z-disc streaming that proceed to severe myofibrillar disintegration, and at later stages, autophagic rimmed vacuoles can be observed [73]. The protein accumulations in human and mouse muscles may be positive for structural proteins (desmin, myotilin, α-actinin, keratin 18) [14,49,88], RNA-binding stress-granule proteins (hnRNPA1, hnRNPA2/B1, TIA1) [49,89,90], TDP-43 [86,88,90], as well as chaperones and cochaperones (HSPA8, CRYAB, HSPB8, SQSTM1, BAG3, STUB1) [14,88,89,95,98]. The rimmed vacuoles are positive for SQSTM1 and the autophagosome marker LC3 (microtubule-associated proteins 1A/1B light chain 3), illustrating their autophagic origin [14,88,95].…”