Analysis of Pathological Activities of CCN2/CTGF in Muscle Dystrophy

Abstract

CCN2 or connective tissue growth factor (CTGF) is a matricellular protein that regulates several cellular processes. In skeletal muscle, CTGF is a key modulator of fibrogenesis, is increased in pathological conditions such as muscular dystrophies, and plays a major role in the pathology outcome. Overexpression of CTGF in skeletal muscle of wild-type mice results in muscle damage, fibrosis, and reduction of strength. In contrast, a decrease in CTGF in dystrophic mice increases strength and reduces damage and fibrosis. Thus, CTGF is a relevant target to study in skeletal muscle pathology and its possible modulation by different treatments or potential new drugs to develop new strategies for the treatment of muscular dystrophies. We summarize the techniques used to detect CTGF in the skeletal muscle of dystrophic mdx mice.