Immunoglobulin therapy modulates the severe inflammatory progression of neuromuscular disorders


The lack of dystrophin in muscle tissue of Duchenne muscular dystrophy (DMD) patients induces a release of DAMPs, CPK and mRNAs in the extracellular environment which interact with toll-like receptors, triggering an innate immune response, with recruitment of inflammasomes and activation of the NF-kB signaling pathway. In addition, muscle antigens are processed and can be presented to T-effector cells, mainly TCD4+ and TCD8+, activating an adaptive immune response characterized by an intensive secretion of pro-inflammatory cytokines and tissue necrosis.


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