(A) Electron microscopy analysis of mitochondria in cultured human MPAN skin fibroblasts (n = 60 mitochondria from 5 neurons). (B-E) Mitochondria respiration assay of cultured human MPAN skin fibroblasts by Seahorse analyzer (B) and quantification of relative basal oxygen consumption rate (OCR) (C), relative maximal OCR (D) and non-mito OCR (E). Results are representative data (Means ± SE) of three independent experiments. Student’s t test and one-way ANOVA followed by Tukey correction, * P < 0.05, ** P < 0.01, *** P < 0.001, n.s., P > 0.05