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Vascular alterations upon activation of TGFbeta signaling in fibroblasts--implications for systemic sclerosis Horn A; Distler JHArthritis Res Ther 2010[]; 12 (3): 125Tissue fibrosis and vascular disease are hallmarks of systemic sclerosis (SSc). Transforming growth factor beta (TGFbeta) is a key-player in fibroblast activation and tissue fibrosis in SSc. In contrast to fibrosis, evidence for a role of TGFbeta in vascular disease of SSc is scarce. Using a transgenic mouse model with fibroblast-specific expression of a kinase-deficient TGFbeta receptor type II, Derrett-Smith and colleagues demonstrate that aberrant TGFbeta signaling in fibroblasts might result in activation of vascular smooth muscle cells and architectural changes of the vessel wall of the aorta.|Animals[MESH]|Aorta/metabolism/pathology[MESH]|Disease Models, Animal[MESH]|Fibroblasts/*metabolism/pathology[MESH]|Mice[MESH]|Mice, Transgenic[MESH]|Muscle, Smooth, Vascular/metabolism/pathology[MESH]|Protein Serine-Threonine Kinases/genetics/metabolism[MESH]|Receptor, Transforming Growth Factor-beta Type II[MESH]|Receptors, Transforming Growth Factor beta/genetics/metabolism[MESH]|Scleroderma, Systemic/*metabolism/pathology[MESH]|Signal Transduction/*physiology[MESH]|Transforming Growth Factor beta/*metabolism[MESH]|Vascular Diseases/*metabolism/pathology[MESH] |
