The fact that these key pathological lesions are also present in skeletal muscle tissue of autosomal‐dominant and ‐recessive human desminopathies [1], [S22] as well as in hetero‐ and homozygous R349P desmin knock‐in mice [6, 25, 27], [S30, S31] underscores the importance of such pathogenetic sequence in desminopathies. The gene discussed is DES; the disease is Desminopathy.