The NDUFV1 constitutes a core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) belonging to the minimal assembly required for catalysis in the transfer of electrons from NADH to the respiratory chain, and has recently been implicated in DCM[37] as well as remodeling in ischemic heart failure[38]. Here, NDUFV1 is linked to familial dilated cardiomyopathy.