Fibrinogen drives dystrophic muscle fibrosis via a TGFbeta/alternative macrophage activation pathway.

@article{Vidal2008FibrinogenDD,
  title={Fibrinogen drives dystrophic muscle fibrosis via a TGFbeta/alternative macrophage activation pathway.},
  author={Berta Vidal and Antonio Laba{\~n}a Serrano and Marc Tjwa and M{\`o}nica Suelves and Esther Ardite and Roberta De Mori and Bernat Baeza-Raja and Maria Martinez de Lagran and Peggy Lafuste and Vanessa Ruiz-Bonilla and Merc{\`e} Sales i Jard{\'i} and Romain Kroum Gherardi and Christo Christov and Mara Dierssen and Peter Carmeliet and Jay L. Degen and Mieke Dewerchin and Pura Mu{\~n}oz-C{\'a}noves},
  journal={Genes & development},
  year={2008},
  volume={22 13},
  pages={
          1747-52
        }
}
In the fatal degenerative Duchenne muscular dystrophy (DMD), skeletal muscle is progressively replaced by fibrotic tissue. Here, we show that fibrinogen accumulates in dystrophic muscles of DMD patients and mdx mice. Genetic loss or pharmacological depletion of fibrinogen in these mice reduced fibrosis and dystrophy progression. Our results demonstrate that fibrinogen-Mac-1 receptor binding, through induction of IL-1beta, drives the synthesis of transforming growth factor-beta (TGFbeta) by mdx… CONTINUE READING

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