Figure 6. Increased iron levels and ferroptosis in MPAN skin fibroblasts.
(A-B) Iron levels were analyzed by cytosolic ferrous probes (A) and Calcein AM (B) in live culture of MPAN skin fibroblasts with C19orf12G58S/G58S or C19orf12p99fs*102 mutations (n = 110 cells). (C) Oxidized lipid levels were analyzed by BODIPY C11 in MPAN skin fibroblasts (n = 110 cells). (D) DFO treatment (50 μM) rescued the cell death caused by erastin in MPAN skin fibroblasts (n = 3 replicates). (E-F) DFO treatment rescued cytosolic ROS levels (E) and ATP levels (F) in MPAN skin fibroblasts (n = 3 replicates). Results are representative data (Means ± SE) of three independent experiments. One-way ANOVA followed by Tukey correction, * P < 0.05, ** P <01. *** p < 0.001, n.s., P > 0.05