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Báo cáo y học: " Vascular alterations upon activation of TGFβ signaling in fibroblasts - implications for systemic sclerosis"

Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học General Psychiatry cung cấp cho các bạn kiến thức về ngành y đề tài: Vascular alterations upon activation of TGFβ signaling in fibroblasts - implications for systemic sclerosis. | Horn and Distler Arthritis Research Therapy 2010 12 125 http arthritis-research.eom content 12 3 125 EDITORIAL Vascular alterations upon activation of TGFP signaling in fibroblasts - implications for systemic sclerosis Angelika Horn and Jorg HW Distler See related research by Derrett-Smith etal. http arthritis-research.eom content 12 2 R69 Abstract Tissue fibrosis and vascular disease are hallmarks of systemic sclerosis SSc . Transforming growth factor p TGFP is a key-player in fibroblast activation and tissue fibrosis in SSc. In contrast to fibrosis evidence for a role of TGFp in vascular disease of SSc is scarce. Using a transgenic mouse model with fibroblast-specific expression of a kinase-deficient TGFp receptor type II Derrett-Smith and colleagues demonstrate that aberrant TGFp signaling in fibroblasts might result in activation of vascular smooth muscle cells and architectural changes of the vessel wall of the aorta. Using the TpRIIAk-fib transgenic mouse model Derrett-Smith and colleagues 1 analyzed a potential role of transforming growth factor p TGFp signaling in the vascular pathogenesis of systemic sclerosis SSc . SSc is a chronic autoimmune disease that affects the skin and various internal organs. The most obvious histopathological alteration of SSc is an extensive accumulation of extracellular matrix 2 . The resulting fibrosis disrupts the physiological tissue structure and frequently leads to dysfunction of the affected organs. The accumulation of extracellular matrix in SSc patients is caused by activated fibroblasts 3 . In addition to fibrosis vascular changes are a major hallmark of SSc. These may be classified into a destructive- and a proliferative vasculopathy. The destructive vasculopathy affects small vessels and manifests early in the course of SSc as progressive loss of capillaries and insufficient angiogenesis. The clinical correlates of the destructive vasculopathy are Raynaud s phenomenon and fingertip ulcers. In contrast Correspondence