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. 2021 Jun 11;53:101271. doi: 10.1016/j.molmet.2021.101271

Figure 2.

Figure 2

SMNKO mice develop severe myopathy from 6 weeks of age.A) Average fiber area in quadriceps muscle of WT and SMNKO mice (n = 2–3). B) Frequency distribution of fiber areas in quadriceps muscle of WT and SMNKO mice (n = 2–3). C) Representative hematoxylin and eosin (HE) staining in quadriceps muscle of WT and SMNKO mice at 8 weeks of age. → Marks central nuclei. ∗ Marks fat replacement. D) Percentage wheat germ agglutinin (WGA) deposition in quadriceps muscle of WT and SMNKO mice (n = 2–3), and representative WGA staining at 8 weeks of age. Bar = 50 μm. E) Quantification of PAX7 positive cells in quadriceps muscle of WT and SMNKO mice (n = 2–3) and representative image at 8 weeks of age. Bar = 50 μm ◄Marks PAX7 positive cells. F) Quantification of Myoblast determination protein 1 (MyoD) positive cells in quadriceps muscle of WT and SMNKO mice (n = 2–3) and representative image at 8 weeks of age. Bar = 50 μm ◄ Marks MyoD positive cells. G) Quantification of central nuclei of quadriceps muscle of WT and SMNKO mice (n = 2–3), and representative cross-section images at 8 weeks of age. Bar = 50 μm ◄ Marks central nuclei. H) Representative cross-sectional image of eMyHC positive cells in 8-week-old SMNKO mice. Bar = 50 μm ∗ Marks normal eMyHC positive fibers after regeneration, ◄ Marks small newly formed eMyHC positive fibers. I) Quantification of F4/80 positive macrophages in quadriceps muscle of WT and SMNKO mice (n = 2–3), and representative image at 8 weeks of age F4/80 and CCL3 double-positive macrophages. Bar = 50 μm ∗ Marks infiltrating F4/80 and CCL3 double-positive macrophages, ◄ marks interstitial F4/80 positive macrophages. Bar graphs: Error bars represent SEM. ∗ Difference to WT control of the same age.