Four out of six analyzed proteases of UPRmt displayed a significant decrease in protein level in DCM and ICM samples compared to control, including CLPP (DCM/Ctrl = 0.59; ICM/Ctrl = 0.63), SPG7 (DCM/Ctrl = 0.66; ICM/Ctrl = 0.55), LONP1 (DCM/Ctrl = 0.54; ICM/Ctrl = 0.51), and OMA1 (DCM/Ctrl = 0.74; ICM/Ctrl = 0.63). Here, SPG7 is linked to familial dilated cardiomyopathy.