Figure 2.

IDB effectiveness correlates with NQO1 protein levels in Ctr and LHON fibroblasts

(A) Western blot analysis and quantification of NQO1 expression in cellular lysates from Ctr (WT1, WT2, and WT3) and LHON (3460/1, 3460/2, 3460/3, 11778/1, 11778/2, and 11778/3) fibroblasts. Actin was used as a loading Ctr. A representative blot of three similar ones is shown. Data are reported as means ± SD of protein abundance of at least three to five independent experiments. Statistical analysis was performed using one-way ANOVA with a Dunnett’s post hoc test comparing the mean of each column with the mean of WT3 as a Ctr column. ∗∗∗p < 0.001.

(B) OCR measurements in Ctr and LHON fibroblasts in the presence or absence of IDB (as in Figure 1B). Open circles correspond to OCR values measured in cells treated with the vehicle DMSO and red squares to OCR values measured in cells treated with 10 μM IDB. Data are reported as mean ± SD of at least three to five independent experiments (Ctr1: n = 5 vehicle, n = 4 IDB; Ctr2: n = 5 vehicle, n = 4 IDB; Ctr3: n = 3 vehicle, n = 3 IDB; 3460/1: n = 3 vehicle, n = 3 IDB; 3460/2, n = 3 vehicle, n = 3 IDB; 3460/3: n = 4 vehicle, n = 3 IDB; 11778/1: n = 3 vehicle, n = 2 IDB; 1177/2: n = 3 vehicle, n = 3 IDB; 11778/3: n = 5 vehicle, n = 4 IDB. Statistical analysis is the same as reported in Figure 1B and STAR Methods. ∗p < 0.05, ∗∗p < 0.01, ∗∗∗p < 0.001.