LONP1 and familial dilated cardiomyopathy: Previously, we showed that many MQC genes, which included UPRmt genes, DDIT3, UBL5, EIF2AK4, HSPA9 (mt-HSP70 protein), HSPE1 (HSP10 protein), YME1L, LONP1, SPG7, HTRA2, and OMA1, were downregulated in the failing myocardium of patients with either ICM or DCM [7].