513 Masaharu Takigawa (ed.), CCN Proteins: Methods and Protocols, Methods in Molecular Biology, vol. 1489, DOI 10.1007/978-1-4939-6430-7_43, © Springer Science+Business Media New York 2017 Chapter 43 Analysis of Pathological Activities of CCN2/CTGF in Muscle Dystrophy María José Acuña and Enrique Brandan 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 condi- tions 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 dif- ferent treatments or potential new drugs to develop new strategies for the treatment of muscular dystro- phies. We summarize the techniques used to detect CTGF in the skeletal muscle of dystrophic mdx mice. Key words CCN2, CTGF, Skeletal muscle, Muscular dystrophy, mdx mice 1 Introduction The matricellular protein CCN2, also known as connective tissue growth factor (CTGF), regulates several cellular processes, such as proliferation, migration, adhesion, and differentiation, and achieves these effects by modulating the activity of various growth factors, such as transforming growth factor type beta (TGF-β), bone mor- phogenetic proteins (BMP), and vascular endothelial growth fac- tor (VEGF), among many others [1, 2]. CTGF participates in the physiopathology of different tissues; in adult skeletal muscle, under normal conditions CTGF is almost absent but is increased in path- ological conditions, such as muscular dystrophies [3, 4], and is a key modulator of muscle fibrogenesis [4, 5]. In this context, over- expression of CTGF in the tibialis anterior of wild-type mice results in muscle damage, fibrosis, and reduction of strength. However, the reduction of CTGF in dystrophic muscle increases strength and reduces damage and fibrosis. Thus, studying CTGF in muscu- lar dystrophies and its possible modulation by different treatments or potential new drugs is a critical issue.