Figure 5.

Effect of P110 treatment on DRP1 and UCP2 expression and energy metabolism in lymphoblasts mutated for the FANC-A gene. (A) WB signals of DRP1 (mitochondrial fission), UCP2 (uncoupling protein 2), and Actin (housekeeping protein used for signals normalization) expression in FAcorr lymphoblasts and FA lymphoblasts treated or not with P110. For all the densitometry graphs, protein expression levels were normalized on the housekeeping signal (Actin), revealed on the same membrane. (B) Energy and lipid metabolism in FAcorr and FA lymphoblasts treated or not with P110: ATP synthesis, oxygen consumption rate (OCR), and P/O value obtained after stimulation with pyruvate/malate (P/M) or succinate (Succ); electron transport between complexes I and III (CI-CIII); cellular lipid content; malondialdehyde (MDA) level, as a marker of lipid peroxidation. All the WB signals reported in (A) are representative of at least three independent experiments. In each panel, data are reported as mean ± SD, and each graph is representative of at least three independent experiments. Statistical significance was tested appropriately with a one-way ANOVA or an unpaired t-test; *, **, ***, and **** represent a significant difference for p < 0.05, 0.01, 0.001, and 0.0001 between FA cells and the FAcorr cells used as control; °, °°, and °°°° represent a significant difference for p < 0.05, 0.01, and 0.0001 between FA cells untreated and treated with P110.