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Therapeutic Potential of Immunoproteasome Inhibition in Duchenne Muscular Dystrophy

10 Aug 2016

Abstract excerpt

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...

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PMID: 27506451
DOI: 10.1038/mt.2016.162

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