Therapeutic Potential of Immunoproteasome Inhibition in Duchenne Muscular Dystrophy.

ABSTRACT: Duchenne muscular dystrophy is an inherited fatal genetic disease characterized by mutations in dystrophin gene, causing membrane fragility leading to myofiber necrosis and inflammatory cell recruitment in dystrophic muscles. The resulting environment enriched in proinflammatory cytokines, like IFN-γ and TNF-α, determines the transformation of myofiber constitutive proteasome into the immunoproteasome, a multisubunit complex involved in the activation of cell-mediate immunity. This event has a fundamental role in producing peptides for antigen presentation by MHC class I, for the immune response and also for cytokine production and T-cell differentiation. Here, we characterized for the first time the presence of T-lymphocytes activated against revertant dystrophin epitopes, in the animal m

SUBMITTER: Farini A 

PROVIDER: S-EPMC5154478 | biostudies-literature | 2016 Nov

REPOSITORIES: biostudies-literature