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. 2015 Jun 5;5:10903. doi: 10.1038/srep10903

Figure 1. Mitochondrial dysfunction in Gaucher fibroblasts.

Figure 1

(a) Mitochondrial enzymatic activities of complex I, II, III and II+III in control and Gaucher fibroblasts was determined as described in Materials and Methods. (b) CoQ levels in control and Gaucher fibroblasts were determined by hexane extraction and HPLC separation as described in Materials and Methods. (c) Mitochondrial membrane potential (ΔΨm) was assessed by flow cytometry using MitoTracker Red. A clear decrease of ΔΨm was observed in Gaucher fibroblasts. (d) ATP levels in control and Gaucher fibroblasts. A significant decrease of ATP levels was observed in Gaucher fibroblasts (e) Representative images of MitoTracker staining in control and Gaucher fibroblasts. Scale bar=15 μm. (f) Magnification of a small area in a Gaucher fibroblast. Red arrow indicates tubular mitochondria with normal polarization. Yellow arrow indicates small mitochondria with a striking depolarization in Gaucher fibroblasts. Scale bar=5 μm. (g) Quantification of depolarized mitochondria in control and Gaucher fibroblasts by fluorescence microscopy determined from 100 randomly selected cells by fluorescence imaging analysis. For control cells, the data are the mean±SD for experiments conducted on 3 different control cell lines. Data represent the mean±SD of 3 separate experiments. *p < 0.05 between control and Gaucher fibroblasts. **p < 0.01 between control and Gaucher fibroblasts.