DES and Desminopathy: Desminopathies (synonyms: desmin-related myopathy, desmin myopathy, desmin storage myopathy, and others [166]) belong to the clinically and genetically heterogeneous group of myofibrillar myopathies (MFM), which are morphologically characterized by the presence of desmin-positive protein aggregates and degenerative changes of the myofibrillar apparatus [160, 164].