Our laboratory seeks to understand the molecular mechanisms that regulate the formation of skeletal muscle and how these are affected in musculoskeletal dystrophies. We have conducted extensive studies to understand the role of the extracellular matrix (ECM) in the formation and regeneration of skeletal muscle and the fibrotic phenomena associated with skeletal muscle dystrophies.

We are interested in understanding the mechanisms involved in fibrosis associated with musculoskeletal diseases. Muscle fibrosis corresponds to an increase in ECM components in skeletal muscle. This occurs, for example, in muscular dystrophies, such as Duchenne muscular dystrophy (DMD), muscle denervation due to loss of motor activity (ALS), or in sarcopenia associated with aging. We have studied the role of profibrotic factors (TGF-beta, CTGF/CCN2), several components of the renin-angiotensin system (RAS), and lysophosphatidic acid (LPA) in the genesis of skeletal muscle fibrosis. Furthermore, we are interested in understanding the cell type(s) responsible for the fibrotic response in different muscle models. We have found several inhibitors of profibrotic factors that are effective in improving muscle pathophysiology and thus correspond to essential proofs of concept in translational medicine. Some of these inhibitors have been used in human patients (in phase 2 and phase 3 clinical trials).