Conversely, knockout of both Oma1 and Yme1l in mouse cardiomyocytes prevents the conversion of L-Opa1 to S-Opa1 forms and restores the standard mitochondrial architecture, in addition to protecting Yme1l mutant mice from cardiomyopathy and early death. The gene discussed is YME1L1; the disease is cardiomyopathy.