Journal: Disease Models & Mechanisms

Article Title: Mitochondrial damage and senescence phenotype of cells derived from a novel frataxin G127V point mutation mouse model of Friedreich's ataxia

doi: 10.1242/dmm.045229

Figure Lengend Snippet: Viability of Fxn G127V MUT MEFs can be rescued by mitigating oxidative stress. (A) Growth analysis of WT, HET and MUT MEFs grown in hypoxic conditions over 6 days in culture. Cells were seeded at equal densities in duplicate wells at day 0, then counted every 24 h. The experiment was repeated twice using two independent MEF lines per genotype ( n =2 biological replicates), and a representative curve is plotted as the mean±s.d. (B) WT, HET and MUT MEF population doubling times as calculated from the growth curves shown in A. Bars represent the mean±s.d.; total measurements per bar=4. (C-E) XTT assays were performed after 24 h treatments of WT and MUT MEFs with respective compounds. Absorbances were recorded (specific A 465 nm and background A 660 nm ), and data for each treatment are expressed relative to untreated WT cells. Each bar represents the mean±s.d. of at least three independent experiments (black dots) performed using two independent MEF lines per genotype ( n =2 biological replicates); total measurements per bar=5 (Omav), 4 (DMF) and 3 (IDB). Significant differences were determined by ordinary one-way ANOVA comparing each treatment with untreated WT (* P <0.05, ** P <0.01, **** P <0.0001).

Article Snippet: Commercially available compounds were obtained as follows: Omav (RTA 408; Cayman Chemical), DMF (Tocris) and idebenone (Sigma-Aldrich).

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