FIGURE 6.

Effect of CoC3 on the expression levels of UPR^mt-related proteins in control and mitochondrial mutant cell lines. Western blot analysis of several UPR^mt -related proteins: Eif2α and P-eif2α proteins as Integrated Stress Response (ISR) markers; ATF4, ATF5 and CHOP proteins as canonical UPR^mt proteins; HSP60 and mtHSP70 proteins as chaperones; SIRT3 protein as main target of pterostilbene; Nrf2 protein as a modulator of mitochondrial antioxidant pathways; NAMPT enzyme as NAD⁺/NADH cell metabolism regulator. “C” stands for healthy control cells. Fibroblasts were treated for 7 days with CoC3 treatment: 1 μM Pterostilbene, 5 μM nicotinamide, 1 μM riboflavin, 1 μM thiamine, 1 μM biotin, 5 μM lipoic acid and 1 μM L-carnitine. Mutations in figure panels: mtND3 (A), NDUFAF6 (B), COX15 (C), NDUFS1 (D), COQ7 (E), NDUFS4 (F). A representative actin band is shown for all assays, although loading control was checked for every Western blot. Band densitometry is shown in Supplementary Figure S7.