In support of this notion, expression of the CDSPs, such as CGAS and ZBP1, and activation of the downstream molecular cascade has been demonstrated in hereditary forms of cardiomyopathy caused by defined mutations in humans and mouse models.39, 40, 41,51,53 Likewise, activation of the nuclear component of the DDR pathway, comprised of the ATM-TP53 axis, has been detected in human hearts and mouse models of heart failure, including hereditary cardiomyopathies.40 Here, ZBP1 is linked to cardiomyopathy.