Figure 4. Relationship between utrophin and fibrosis.

Halofuginone at a concentration of 7.5 μg/mouse was injected IP into male mdx mice three times/week for 10 weeks, starting at 4 weeks of age. The diaphragms were double-immunostained for utrophin (red) with collagen type I (green, upper panel) or cthrc1 antibodies (green, lower panel). Nuclei were stained with DAPI (blue). In the mdx mice, areas with high levels of collagen (upper panel) or cthrc1 (lower panel) and low levels of utrophin were observed (separated by the dashed lines). Visualization was clearer in the 3D reconstruction. Halofuginone treatment decreased the levels of both collagen type I and cthrc1, whereas the utrophin level increased.