Figure 1.

Mitochondrial functions of Lonp1 in myocytes and cardiomyocytes. In mitochondria, Lonp1 (i) regulates mitophagy through Pink1 degradation; (ii) regulates protein turnover by degrading misfolded or damage proteins; (iii) modulates mitochondrial dynamics through the action of Mfn1 and Mfn2, which promote fusion, and Drp1, which promotes fission; (iv) regulates stress response by preserving mtDNA integrity and modulating ROS production; (v) modulates respiratory chain complexes activity.