In line with human patients with HSPB8-myopathy, this myopathy resembles human myofibrillar myopathy with Z-disc disorganization, rimmed vacuoles, protein aggregates containing HSPB8, αB-crystallin, and desmin, as well as accumulation of LC3 and SQSTM1/p62 suggestive of possible impairment in the autophagic flux [60]. Here, DES is linked to myopathy.