COPS8 and dilated cardiomyopathy: Mice with cardiac-specific deletion of Csn8 have increased total neddylated proteins and develop cardiac hypertrophy, dilated cardiomyopathy and eventually died from heart failure by age of 4 weeks.301 Furthermore, tamoxifen-induced Csn8 knockout in the adult heart induces rapid heart failure and mice die within 2 weeks after induction.302 Thus, the CSN8-mediated deneddylation is required in the maintenance of cardiac integrity in both postnatal and adult hearts.