In line with human patients with HSPB8-related myopathy, the muscle pathology shows features of human myofibrillar myopathy, with Z-disc disintegration, accumulation of granulofilamentous material, aggregates positive for HSPB8, CRYAB, and desmin, and rimmed vacuoles [443]. The gene discussed is HSPB8; the disease is myofibrillar myopathy.