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. 2017 Dec 19;6:e30054. doi: 10.7554/eLife.30054

Figure 4. Cardiopathology of frataxin knockdown mice.

(a) Gomori’s iron staining and quantification of iron deposition in dox treated transgenic (Tg +), wild-type (Wt +) and dox withdrawn transgenic (Tg ± Rescue) animals. Dox treated transgenic (Tg +) mice showing myocardial iron-overload (a) also displayed altered expression of ferritin protein (b) which is involved in iron storage. Both iron-overload and ferritin protein levels were significantly lower in Tg ± Rescue animals (a–b). (c) Masson's trichrome staining and quantification showing increased fibrosis in Tg + mice when compared to Wt + and Tg ± Rescue animals. (d) Electron micrographs of cardiac muscle from Wt +, Tg + and Tg ± Rescue animals at 20 week after dox treatment. Double arrow lines indicate sarcomere. m = mitochondria. Scale bars, 1 μm. Data are representative of three biological replicates per group. (e) Higher magnification of electron micrographs of cardiac muscle from Tg + mice, showing normal (m) and degenerating mitochondria (asterisks). (f) Aconitase activity was assayed in triplicate in tissues removed from three hearts in each group. Values represent mean ±SME. One-way ANOVA test *p≤0.05.

Figure 4—source data 1. This spreadsheet contains the raw signal intensity quantification data of ferric iron, ferritin and collagen staining which was used to generate the graphs shown in Figure 4a–c and the aconitase and citrate synthase enzymatic activity measurements are provided (Figure 4f).
elife-30054-fig4-data1.xlsx (90.7KB, xlsx)
DOI: 10.7554/eLife.30054.014

Figure 4.

Figure 4—figure supplement 1. Frataxin deficiency in mouse heart results in iron accumulation and increased levels of ferritin and ferroportin.

Figure 4—figure supplement 1.

(a) Gomori’s iron staining in sections of heart from Frataxin knockdown mice (Tg +) shows iron accumulation (insert) but not in wild type mice (Wt +). Scale bar represents 100 μm. (b–c) Immunohistochemical staining of heart from doxycycline treated wild type (WT) and Fxn knockdown (TG) mice using anti-rabbit ferritin (b) and ferroportin (c) antibodies. Original magnification: 20x.