Similar to plectin-null, MCK-Cre/cKO, P1-, P1b-, and P1d-KO mice, muscle specimens from these patients showed myofibrillar degeneration, disruption of the desmin networks with formation of desmin aggregates, clustering of nuclei, mitochondrial abnormalities, and symptoms of cardiomyopathy. The gene discussed is DES; the disease is cardiomyopathy.