Desminopathy is among the myofibrillar myopathies (MFMs), which are hereditary muscle disorders characterized by specific histopathological features of protein aggregation; in particular, focal disintegration of myofibrils predominantly at the Z-disc level and sarcoplasmic protein aggregates that show an accumulation of several proteins, among which is desmin [3,50]. Here, DES is linked to muscular disease.