To unravel the underlying cause of this mitochondrial dysfunction, we subsequently analyzed left ventricle (LV) samples from patients with dilated cardiomyopathy (DCM), as well as from muscle LIM protein (Mlp)-deficient (Mlp−/− or Csrp3−/−) mice, which develop eccentric hypertrophy comparable to human DCM due to alterations in the cytoarchitecture of cardiomyocytes29. This evidence concerns the gene CSRP3 and familial dilated cardiomyopathy.