CLDN5 and cardiomyopathy: Supporting this, Cldn5 was significantly downregulated at the cardiomyocyte lateral membrane in a dystrophin–utrophin double knockout (Dmdmdx;Utrn−/− dko) mouse model that recapitulates features of cardiomyocyte degeneration and myocarditis, implicating Cldn5 loss in cardiomyopathy pathogenesis [135].