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Logo of arthrestherBioMed Centralbiomed central web sitesearchsubmit a manuscriptregisterthis articleArthritis Research & Therapy
Arthritis Res Ther. 2010; 12(3): 125.
Published online Jun 18, 2010. doi:  10.1186/ar3026
PMCID: PMC2911883
Vascular alterations upon activation of TGFβ signaling in fibroblasts - implications for systemic sclerosis
Angelika Horn1 and Jörg HW Distlercorresponding author1
1Department of Internal Medicine III and Institute for Clinical Immunology, University of Erlangen-Nuremberg, 91054 Erlangen, Germany
corresponding authorCorresponding author.
Angelika Horn: angelika.horn/at/; Jörg HW Distler: joerg.distler/at/
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.
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