Figure 1

Quantification of fibrosis in human dystrophic muscle. (A) Representative Sirius red staining of healthy and dystrophic human muscle sections reveals the extent of collagen deposition in patients with Duchenne muscular dystrophy (DMD). (B) Percentage of fibrosis (collagen content) in healthy and DMD muscles as measured by Sirius red staining in muscle sections. Data correspond to the mean ± SEM; n = 6 for DMD group and n = 5 for control group. Non-parametric Mann–Whitney U test was used for comparison. **P <0.01 versus healthy controls. (C) Active transforming growth factor beta 1 (TGFβ1) protein levels measured by ELISA in muscle biopsy material from healthy and DMD muscle. Data correspond to the mean ± SEM; n = 5 on each group. Non-parametric Mann–Whitney U test; **P <0.01 versus healthy controls. (D) Immunohistochemistry for phosphorylated-Smad2/3 (P-Smad2/3) in healthy and dystrophic human muscle sections. (E) Quantitative RT-PCR for collagen I (Coll 1), fibronectin (FN), tissue inhibitor of metalloproteinases 1(TIMP-1), TGFβ1 and connective tissue growth factor (CTGF) in DMD muscles compared to healthy muscles (which were given the arbitrary value of 1). Data correspond to the mean ± SEM; n = 4 on each group. Non-parametric Mann–Whitney U test *P <0.05. Scale bars = 50 μm.