In line with these findings, heart-specific nexilin knockout mouse models, generated by breeding Nexnfl/fl mice with either cardiomyocyte-specific Xenopus laevis myosin light-chain 2 (XMLC2)-Cre transgenic mice or with cardiomyocyte-specific troponin T2-Cre transgenic mice developed similar phenotypes: well-organized heart architecture at the embryonic stage and rapid progressive DCM with early death before P12. Here, NEXN is linked to familial dilated cardiomyopathy.