Mouse Models of Muscle Fibrosis.

Abstract

Fibrosis in skeletal muscle is the natural tissue response to persistent damage and chronic inflammatory states, cursing with altered muscle stem cell regenerative functions and increased activation of fibrogenic mesenchymal stromal cells. Exacerbated deposition of extracellular matrix components is a characteristic feature of human muscular dystrophies, neurodegenerative diseases affecting muscle and aging. The presence of fibrotic tissue not only impedes normal muscle contractile functions but also hampers effective gene and cell therapies. There is a lack of appropriate experimental models to study fibrosis. In this chapter, we highlight recent developments on skeletal muscle fibrosis in mice and expand previously described methods by our group to exacerbate and accelerate fibrosis development in murine muscular dystrophy models and to study the presence of fibrosis in muscle samples. These methods will help understand the molecular and biological mechanisms involved in muscle fibrosis and to identify novel therapeutic strategies to limit the progression of fibrosis in muscular dystrophy.