Research ArticleMuscular Dystrophy

Regulatory T cells suppress muscle inflammation and injury in muscular dystrophy

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Science Translational Medicine  15 Oct 2014:
Vol. 6, Issue 258, pp. 258ra142
DOI: 10.1126/scitranslmed.3009925

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Tregs Muscle In on the Action

Duchenne muscular dystrophy (DMD) is caused by mutations in the dystrophin gene leading to muscle degeneration. Villalta et al. now show that the imbalance between proinflammatory and proregulatory immune cells in both humans with DMD and in the mdx mouse model of the disease is directly involved in myofiber damage. Increasing the number of anti-inflammatory regulatory T cells (Tregs) in dystrophic muscle prevents tissue destruction and ameliorates clinical manifestations. Treg elimination resulted in increased production of proinflammatory cytokines and macrophages, whereas treatment with the Treg-promoting cytokine interleukin-2 (IL-2) increased immunosuppressive IL-10 production and resolved myofiber damage. Thus, this study shows that Tregs modulate the progression of muscular dystrophy by suppressing type 1 inflammation and highlight the potential of Treg-modulating agents as DMD therapeutics.

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