Figure 4.

Complex I deficiency leads to altered mitochondrial function in LHON-affected individuals, which is compensated in carriers

(A–D) Measurements of mitochondrial ROS production in LHON-derived 3460 fibroblasts (A) and cybrids (B) and in 11778 fibroblasts (C) and cybrids (D) by using MitoSOX red probe.

(E–H) The mitochondrial transmembrane potential (Ψ_m) of 3460 and 11778 fibroblasts and cybrids was detected by using the Ψ_m-sensitive probe TMRM. When indicated, the cells were STARV. for 30 min before TMRM loading.

Data are presented as means ± SEM. n = at least 5 visual fields per at least 3 independent samples per condition. ^∗p < 0.05, ^∗∗p < 0.01, and ^∗∗∗p < 0.001.