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Cited by 257

The DOI matches its Crossref record; 5 of the 5 references it cites were found in a registry or an index.

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

Open access
Vidal, Berta, Serrano, Antonio L., Tjwa, Marc, Suelves, Mònica, Ardite, Esther, De Mori, Roberta, Baeza-Raja, Bernat, Martínez de Lagrán, María, Lafuste, Peggy, Ruiz-Bonilla, Vanessa, Jardí, Mercè, Gherardi, Romain, Christov, Christo, Dierssen, Mara, Carmeliet, Peter, Degen, Jay L., Dewerchin, Mieke, Muñoz-Cánoves, Pura·2008·Genes & Development

Abstract

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-1β, drives the synthesis of transforming growth factor-β (TGFβ) by mdx macrophages, which in turn induces collagen production in mdx fibroblasts. Fibrinogen-produced TGFβ further amplifies collagen accumulation through activation of profibrotic alternatively activated macrophages. Fibrinogen, by engaging its αvβ3 receptor on fibroblasts, also directly promotes collagen synthesis. These data unveil a profibrotic role of fibrinogen deposition in muscle dystrophy.

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Metadata

Authors
Vidal, B., Serrano, A. L., Tjwa, M., Suelves, M., Ardite, E., De Mori, R., Baeza-Raja, B., Martínez de Lagrán, M., Lafuste, P., Ruiz-Bonilla, V., Jardí, M., Gherardi, R., Christov, C., Dierssen, M., Carmeliet, P., Degen, J. L., Dewerchin, M., & Muñoz-Cánoves, P.
Venue
Genes & Development
Published
2008-07-01
Volume
22
Issue
13
Pages
1747-1752
ISSN
  • 0890-9369 (ISSN-L)
  • 1549-5477
OpenAlex
W2150697205
Cited by
257
References
5
Type
posted content
Language
en
Open access
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CoChat. (2026). Citation check: Fibrinogen drives dystrophic muscle fibrosis via a TGFβ/alternative macrophage activation pathway. https://app.cochat.ai/papers/10.1101/gad.465908
The paper
Vidal, B., Serrano, A. L., Tjwa, M., Suelves, M., Ardite, E., De Mori, R., Baeza-Raja, B., Martínez de Lagrán, M., Lafuste, P., Ruiz-Bonilla, V., Jardí, M., Gherardi, R., Christov, C., Dierssen, M., Carmeliet, P., Degen, J. L., Dewerchin, M., & Muñoz-Cánoves, P. (2008). Fibrinogen drives dystrophic muscle fibrosis via a TGFβ/alternative macrophage activation pathway. Genes & Development. https://doi.org/10.1101/gad.465908

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.