Figure 7.

NK1 rescues mitochondrial morphology in MPS IIIB patient fibroblasts

Control HDFa and MPS IIIB patient fibroblasts were grown on coverslips and treated or not (mock) with NK1 10⁻⁶ M for 48 h before being incubated with MitoTracker. Single focal sections are shown. Images are representative of three independent experiments made in triplicates. Scale bar: 50 μm. The histograms on the right show the quantification relative to the percentage of cells showing elongated mitochondria as mean value of fluorescence intensity (pink bars). Means ± SEM were obtained from three independent experiments. ∗∗∗ p value <0.001. ns = not significant.