DES and Desminopathy: In desminopathies, faulty desmin expression inflicts a multitude of aberrations, thereby negatively affecting the mechanical stability of muscle cells, myofibrillar spatial organization with subsequently impaired force generation, and the structure and function of intercalated discs, neuromuscular junctions, and the mitochondrial network [4,8,11,12,14,23,24,25,26,27,28,29,30,31,32,33,34,35].