Fig. 6
From: Mitochondrial modulation with leriglitazone as a potential treatment for Rett syndrome
Effect of leriglitazone in fibroblasts from primary mitochondrial diseases. Given LGZ mechanism of action we investigated its potential effect on the treatment of mitochondrial diseases. For that, we selected fibroblasts from a patient bearing mutations in NDUFS1, which affect the bioenergetic function. A Non-glycolytic ATP concentration detected by luciferin-luciferase luminescence upon incubation with 2-deoxyglucose, and B O2− generation—detected with MitoSOX probe by flow cytometry—were evaluated, confirming the effect of leriglitazone in mitochondrial patients. All experiments were done in at least two technical replicates and each experiment was done in triplicates. Statistical analyses were performed as described in Materials and Methods. *p < 0.05, **p < 0.01, ****p < 0.0001. Absence of asterisk means no statistically significant difference. Control fibroblasts in grey; NDUFS1-patient’s fibroblasts in blue; LGZ-treated NDUFS1-patient’s fibroblasts in rouge