Tissue Engineering and Regenerative Medicine

Duchenne muscular dystrophy (DMD) is characterized by absence of the protein dystrophin, muscle wasting and fibrosis.We have evaluated the role of the profibrotic connective tissue growth factor (CTGF/CCN2) in a genetically model (mdx-CTGF+/- mice) and in a decreasing CTGF activity model, by blocking CTGF antibodies in mdx mice. In both models, we observed a decrease of fibrosis together with a significant less muscle damage compared to mdx mice. The decrease or blocking of CTGF caused an improvement of muscle strength and improvement of wild type cell graft. Thus, CTGF could be novel therapeutic agents to treat muscle fibrosis associated to DMD and improve cell therapeutic approaches.