Figure 3 | Scientific Reports

Figure 3

From: Pharmacological Chaperones and Coenzyme Q10 Treatment Improves Mutant β-Glucocerebrosidase Activity and Mitochondrial Function in Neuronopathic Forms of Gaucher Disease

Figure 3

ROS production in Gaucher fibroblasts.

(a) Mitochondrial ROS levels in control and Gaucher fibroblasts. Results are expressed as the ratio of MitoSOX signal to 10-N-nonyl acridine orange signal. (b) H2O2 levels in control and Gaucher fibroblasts by CMH2-DCFDA staining coupled with flow cytometry analysis. (c) Mitochondrial ROS levels in control and Gaucher-B fibroblasts cultured, in the absence or presence of CoQ (25 μM), PC NAdBT-AIJ (25 μM) or the combination of both CoQ + PC (25 μM + 25 μM) for 96 h. (d) H2O2 levels in control and Gaucher-B fibroblasts cultured, in the absence or presence of CoQ (25 μM), PC NAdBT-AIJ (25 μM) and the combination of both CoQ + PC (25 μM + 25 μM) for 96 h. The mean ± SD of 3 independent experiments are showed. *p < 0.01 between control and Gaucher fibroblasts. ap < 0.05 between the presence and the absence of CoQ. bp < 0.05 between the presence and the absence of PC. cp < 0.05 between the presence and the absence of CoQ + PC. #p < 0.05 between CoQ + PC and CoQ or PC treatment.

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