LONP1 and cardiomyopathy: In this model, Lonp1 and caseinolytic protease proteolytic subunit (ClpP), another mitochondrial matrix protease involved in protein quality control, resulted in upregulation at mid-stage cardiomyopathy if compared to control mice [89], with a concomitant reduction in the levels of Fe-S cluster proteins, including aconitase, NDUFS3 of Complex I, SDHB of Complex II, and Rieske protein of Complex III.