Scar wars is TGFβ the phantom menace in scleroderma 英文参考文献.docVIP

Available online /content/8/4/213 Review Scar wars: is TGFβ the phantom menace in scleroderma? Andrew Leask Division of Oral Biology and Department of Physiology and Pharmacology, CIHR Group in Skeletal Development and Remodeling, Division of Oral Biology, Schulich School of Medicine and Dentistry, University of Western Ontario, Dental Sciences Building, London, ON N6A 5C1, Canada Corresponding author: Andrew Leask, Andrew.Leask@schulich.uwo.ca Published: 9 June 2006 Arthritis Research Therapy 2006, 8:213 (doi:10.1186/ar1976) This article is online at /content/8/4/213 ? 2006 BioMed Central Ltd Abstract fibrotic responses, including SSc [5]; however, the exact contribution of TGFβ to the fibrotic phenotype of SSc is unclear. Furthermore, as TGFβ plays many roles in normal The autoimmune disease scleroderma (systemic sclerosis (SSc)) is characterized by extensive tissue fibrosis, causing significant morbidity. There is no therapy for the fibrosis observed in SSc; indeed, the underlying cause of the scarring observed in this disease is unknown. Transforming growth factor-β (TGFβ) has long been hypothesized to be a major contributor to pathological fibrotic diseases, including SSc. Recently, the signaling pathways through which TGFβ activates a fibrotic program have been elucidated and, as a consequence, several possible points for anti-fibrotic drug intervention in SSc have emerged. physiology, including as a suppressor of the immune response and epithelial proliferation, broadly targeting TGFβ signaling for the treatment of disease is anticipated to be problematic [8]. Thus, much interest exists, from both clinical and pharmaceutical points of view, in identifying methods of intervening within the TGFβ signaling cascade in such a fashion that the pro-fibrotic aspects of TGFβ signaling are blocked but other TGFβ-dependent processes are unaltered.