Since the cardiac-specific DN-VCP TG mouse exhibits a phenotype of chronic dilated cardiomyopathy similar to that observed in IBMPFD and other idiopathic DCM, this mouse model provides a unique tool to investigate the molecular mechanisms of cardiomyopathy involved in these complex human disorders, holding great promise for exploring therapeutic targets and thus offering a significant clinical relevance. The gene discussed is VCP; the disease is familial dilated cardiomyopathy.