A major drawback of our DKI mice, the model for the autosomal-recessive desminopathies with maintained desmin protein expression, is the late-onset and rather mild form of dilated cardiomyopathy, [21] which is highlighted by the values of the left-ventricular FS and posterior wall thickness that were in the same range as in the WT during the observation time (Fig. 5a). The gene discussed is DES; the disease is Desminopathy.