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- W2117979247 abstract "In Duchenne muscular dystrophy (DMD), a persistently altered and reorganizing extracellular matrix (ECM) within inflamed muscle promotes damage and dysfunction. However, the molecular determinants of the ECM that mediate inflammatory changes and faulty tissue reorganization remain poorly defined. Here, we show that fibrin deposition is a conspicuous consequence of muscle-vascular damage in dystrophic muscles of DMD patients and mdx mice and that elimination of fibrin(ogen) attenuated dystrophy progression in mdx mice. These benefits appear to be tied to: (i) a decrease in leukocyte integrin αMβ2-mediated proinflammatory programs, thereby attenuating counterproductive inflammation and muscle degeneration; and (ii) a release of satellite cells from persistent inhibitory signals, thereby promoting regeneration. Remarkably, Fib-gamma(390-396A) (Fibγ390-396A) mice expressing a mutant form of fibrinogen with normal clotting function, but lacking the αMβ2 binding motif, ameliorated dystrophic pathology. Delivery of a fibrinogen/αMβ2 blocking peptide was similarly beneficial. Conversely, intramuscular fibrinogen delivery sufficed to induce inflammation and degeneration in fibrinogen-null mice. Thus, local fibrin(ogen) deposition drives dystrophic muscle inflammation and dysfunction, and disruption of fibrin(ogen)-αMβ2 interactions may provide a novel strategy for DMD treatment." @default.
- W2117979247 created "2016-06-24" @default.
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- W2117979247 date "2012-03-01" @default.
- W2117979247 modified "2023-10-14" @default.
- W2117979247 title "Amelioration of Duchenne muscular dystrophy in mdx mice by elimination of matrix-associated fibrin-driven inflammation coupled to the αMβ2 leukocyte integrin receptor" @default.
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- W2117979247 doi "https://doi.org/10.1093/hmg/dds012" @default.
- W2117979247 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3315206" @default.
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