Nintedanib as a new therapeutic agent for Duchenne muscular dystrophy: preclinical in vitro and in vivo studies


Several growth factors have been involved in the process of muscle fibrosis in Duchenne muscular dystrophy (DMD). Nintedanib, a tyrosine kinase inhibitor, is effective in fibrotic disorders such as idiopathic lung fibrosis. We studied whether nintedanib could be effective slowing down fibrosis in DMD. We studied the influence of nintedanib on proliferation, chemotaxis and gene expression of human fibroblasts in vitro. We studied the effect of nintedanib in muscle function and structure in 10 months old mdx treated-mice (n = 7) with 60 mg/kg nintedanib daily for one month compared to age matched mdx mice (n = 5) and C57BL/6N controlmice (n = 5) using digigait, electromyography and histological studies.


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