Immune-mediated Mechanisms and Immunotherapy of Duchenne Muscular Dystrophy
Journal Title: Journal of Musculoskeletal Disorders and Treatment - Year 2016, Vol 2, Issue 1
Abstract
The immune response is a critical mechanism for dystrophic muscle pathology and muscle wasting in Duchenne Muscular Dystrophy (DMD). The inflammatory processes are supposed to associate with activation of the innate immune response like macrophages infiltration, T cell-mediated immune response, generation of cytokines and chemokines and the aberrant activation of NF-κB signaling pathway, which contribute to severe muscle necrosis and fibrosis. Immunotherapy has been developed to manage DMD, and glucocorticoids are used to treat DMD patients due to their anti-inflammatory functions. Furthermore, immune targeting therapies and Mesenchymal stem cells (MSCs) transplantation could be promising therapeutic approaches in the future.
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