ADAMTS-5 inhibition reduces muscle inflammation and fibrosis and improves function in mouse models of Duchenne muscular dystrophy

mice, GLPG1972 improved forelimb grip strength and wire hang time compared with vehicle. Furthermore, GLPG1972 increased the strength of fast tibialis anterior muscles in situ and slow soleus muscles ex vivo, and this was associated with decreased inflammation, sarcolemma damage, and fibronectin deposition as assessed by immunohistochemistry. Together, these findings position ADAMTS-5 inhibition with GLPG1972 for further study as a disease-modifying strategy for DMD that may achieve both anti-inflammatory and antifibrotic effects on muscle, resulting in improved contractile function.