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Editorial

Vascular alterations upon activation of TGFβ signaling in fibroblasts - implications for systemic sclerosis

Angelika Horn and Jörg HW Distler*

Author affiliations

Department of Internal Medicine III and Institute for Clinical Immunology, University of Erlangen-Nuremberg, 91054 Erlangen, Germany

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Citation and License

Arthritis Research & Therapy 2010, 12:125  doi:10.1186/ar3026


See related research by Derrett-Smith et al., http://arthritis-research.com/content/12/2/R69

Published: 18 June 2010

Abstract

Tissue fibrosis and vascular disease are hallmarks of systemic sclerosis (SSc). Transforming growth factor β (TGFβ) is a key-player in fibroblast activation and tissue fibrosis in SSc. In contrast to fibrosis, evidence for a role of TGFβ in vascular disease of SSc is scarce. Using a transgenic mouse model with fibroblast-specific expression of a kinase-deficient TGFβ receptor type II, Derrett-Smith and colleagues demonstrate that aberrant TGFβ signaling in fibroblasts might result in activation of vascular smooth muscle cells and architectural changes of the vessel wall of the aorta.