Figure 1

Increased mitochondrial membrane potential in fibroblasts derived from patients with IPD. (A) Analysis performed at baseline respectively after FCCP perturbation at the single-cell level. The histograms show the mean TMRM fluorescence in the cell area. Statistics: Wilcoxon rank-sum test, ***indicates P < 0.001. Counts of analysed cells per group: Ctrl unperturbed, 22.136; IPD unperturbed, 28.005; IPD + FCCP, 29.811; Ctrl + FCCP, 20.789. (B) Analysis performed at baseline at the level of individual subjects. Individual analysis at baseline condition showed no significant difference in TMRM fluorescence between patients and controls (P = 0.065). (C) Analysis performed after FCCP perturbation at the individual subject level. The difference in TMRM fluorescence between patients with IPD and controls was significant. Statistics: Wilcoxon rank-sum test, ***indicates P < 0.001. (D) Analysis of correlation between pairwise features. Pearson coefficients of correlation are provided as white text and color-coded. Dendrograms illustrate hierarchical clustering with Euclidean distance metric and average linkage. Correlations between cellular doubling time and cellular morphological parameters were negligible (correlation coefficient |r| < 0.30). Furthermore, mostly negligible (|r| < 0.30) and one low (0.30 < |r| < 0.50) but no moderate (0.50 < |r| < 0.70) or higher correlations were found between doubling time and functional or morphometric mitochondrial features.