Figure 2.

SMT022357 increases utrophin expression in skeletal muscles. (A) Immunofluoresence staining for utrophin in EDL and SOL muscles of 7 weeks old mdx mice treated for 5 weeks with 30 mg/kg/day SMT022357 or vehicle. Transverse sections were stained with anti-utrophin polyclonal antibody URD40 and anti-rabbit secondary antibody. Scale bar: 100 µm. (B) Longitudinal cryosections of TA muscle following treatment of mdx mice homogenously increased the utrophin expression along the myofiber after treatment with SMT022357 compared to vehicle group. Scale bar: 100 µm. (C) Co-immunohistochemical staining of TA muscle with utrophin, α-Bungarotoxin and DAPI prepared from mdx mice treated with SMT022357. White arrows indicate utrophin expression in extra-synaptic regions of the sarcolemma. Scale bar: 50 µm.