Recently, studies in Lrrc10 knockout mice (Brody et al., 2012, 2016) and the identification of LRRC10 mutations in human DCM (Qu et al., 2015) have sparked interest in the underlying molecular mechanisms that mediate cardiac disease when LRRC10 is absent or mutated. This evidence concerns the gene LRRC10 and familial dilated cardiomyopathy.