Fig 1: H2O2 and Insulin exacerbate defective mitochondrial proteostasis in PITRM1MUT fibroblasts. (A) Representative western blot analysis of PITRM1, IDE, PMPCB, and Frataxin (FXN) in fibroblasts derived from a control (CTRL M) and patient 1 (PT1) exposed to H2O2 and Insulin for 48 h. Densitometric evaluations expressed as fold change compared to untreated CTRL M are reported in (B), GAPDH was used as loading control. (C) TMRM staining in CTRL M and PT1 exposed to H2O2 or Insulin; magnification 40×. (D) Quantitative analysis of mitochondrial morphology (elongated versus fragmented mitochondria) in each experimental setting shown in (C). Colour codes as in (B). Statistical analyses were performed, for each gene, with RM-1-way Anova followed by Fisher LSD test (C), or with 2-way Anova followed by Sidak’s multiple comparisons test (D); *p < 0.05, **p < 0.005.