期刊
EXPERT REVIEW OF CLINICAL IMMUNOLOGY
卷 7, 期 4, 页码 491-498出版社
TAYLOR & FRANCIS LTD
DOI: 10.1586/ECI.11.39
关键词
collagen synthesis; fibroblasts; fibrosis; SMAD; systemic sclerosis; TGF-beta
类别
资金
- Fondo de Investigacion Santitaria del ISCIII PI (Spain) [08/0316]
Systemic sclerosis (SSc) is a chronic systemic disease characterized by autoimmunity, vascular lesions and progressive fibrosis. The fibrotic component is dominant in SSc compared with other vascular or autoimmune diseases and determines its prognosis and therapeutic refractoriness. Fibroblasts are responsible for abnormal extracellular matrix accumulation. Studies in cultured SSc skin fibroblasts have facilitated the identification of potential pathways involved in their profibrotic phenotype. Profibrotic fibroblasts characterized by abnormal growth and extracellular matrix synthesis may differentiate or expand from normal resident fibroblasts. Recruitment of bone marrow-derived progenitors and transdifferentiation of different cell lineages might also be involved. Multiple factors and signaling pathways appear to be involved in the development or persistence of the SSc fibroblast phenotype. Although their relative relevance and interplay are unclear, aberrant TGF-beta signaling seems pivotal and constitutes the best characterized therapeutic target.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据