Next, we investigated the effects of NV118 on cellular ROS production in control and fibroblast cells modeling LS, and harboring pathogenic mtDNA variants (T8993G, T10158C, and T12706C), in MTATP6, MTND3, and MTND5 genes leading to complex V and I defect and depressed ATP production. This evidence concerns the gene MT-ATP6 and Leigh syndrome.