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. 2023 Feb 17;12:e75825. doi: 10.7554/eLife.75825

Figure 1. Iron restriction delays mitochondrial disease in mice.

(A) Correlation between the onset of clasping and survival. Each point represents data from a single mouse. p=0.0047, Pearson’s test. (B) Age at which Ndufs4−/− mice exhibited the clasping phenotype on chow diet. Mice were treated with either vehicle or deferiprone (DFP) in the water (2 mg/mL) from weaning. (C) Survival curves of Ndufs4−/− mice fed a chow diet and treated with deferiprone in the water (2 mg/mL) from weaning. (D) Onset of clasping in Ndufs4−/− mice on AIN-93G synthetic diet containing normal (40 ppm, con) or low (8 ppm) iron starting from weaning. Mice on control diet (40 ppm, Fe) were also treated with iron-dextran (100 mg/kg every 3 days via i.p. injection, high) from weaning. (E) Survival curves of mice on normal (40 ppm) or low (8 ppm) AIN-93G synthetic diet. (F) Weight gain in wild-type (WT, square markers) or Ndufs4−/− mice (KO, circle markers) on AIN-93G synthetic diet containing normal (40 ppm, red) or low (8 ppm, blue) concentrations of iron. p Value was calculated by log-rank for lifespan analyses. ****p<0.0001, t test with Bonferroni Correction.

Figure 1.

Figure 1—figure supplement 1. Brain-permeable iron chelators are effective in delaying mitochondrial disease.

Figure 1—figure supplement 1.

(A) Age at which Ndufs4−/− mice exhibited the clasping phenotype on chow diet. Mice were treated with either vehicle, deferiprone (DFP) in the water (0.5 or 1 mg/mL), or deferoxamine (DFO) via daily i.p. injection (125 mg/kg) from weaning. (B) Survival curves of Ndufs4−/− mice fed a chow diet and treated with deferiprone in the water (0.5 mg/mL) from weaning. (C) Survival curves of Ndufs4−/− mice fed a chow diet and treated with deferiprone in the water (1 mg/mL) from weaning. (D) Survival curves of Ndufs4−/− mice fed a chow diet and treated with deferoxamine via daily i.p. injection (125 mg/kg) from weaning. p Value was calculated by log-rank for lifespan analyses. ****p<0.0001, t test.
Figure 1—figure supplement 2. Iron restriction induces iron-deficiency anemia.

Figure 1—figure supplement 2.

Complete blood count in PND30 WT or Ndufs4−/− mice on normal (40 ppm) or low (8 ppm) AIN-93G synthetic diet to quantify (A) hematocrit, (B) hemoglobin, (C) red blood cell count which were used to calculate (D) mean corpuscular volume, (E) mean corpuscular hemoglobin, and (F) mean corpuscular hemoglobin concentration.
N=3–6 mice. **p<0.01, ***p<0.001, ****p<0.0001, t test. WT, wild-type.