Role of endoglin in fibrosis and scleroderma.
Role of endoglin in fibrosis and scleroderma.
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DOI：
10.1016/b978-0-12-394308-8.00008-x
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发表时间：
2012
影响因子：
--
通讯作者：
ten Dijke, Peter
中科院分区：
文献类型：
--
作者：
Maring, Janita A.;Trojanowska, Maria;ten Dijke, Peter
纤维化在许多病理状况中起作用，其中之一是自身免疫性疾病系统性硬化症（SSc）。SSc的特征是皮肤和内脏器官的纤维化，但病因仍有待阐明。转化生长因子-β（TGF-β）是纤维化过程中的关键参与者，也在SSc中。TGF-β诱导细胞外基质的几种组分的产生，并诱导成纤维细胞分化为肌成纤维细胞，这进一步促进纤维化。虽然TGF-β在纤维化中已被广泛研究，但其信号通路的几个组分的作用仍然未知。内皮糖蛋白是TGF-β的共受体，并且已知调节TGF-β信号传导。因此，内皮糖蛋白可以增强TGF-β在纤维化中的作用或作为抑制剂。已经进行了多项研究，支持这两种假设。阐明内皮糖蛋白在纤维化过程中TGF-β信号传导中的确切作用对于理解纤维化过程非常重要，并可能导致更好的治疗方法的发展。
Fibrosis plays a role in many pathological conditions, among which is the autoimmune disease systemic sclerosis (SSc). SSc is characterized by fibrosis in the skin and internal organs, but the etiology remains to be elucidated. Transforming growth factor-β (TGF-β) is a key player in the fibrotic process, also in SSc. TGF-β induces the production of several components of the extracellular matrix and induces differentiation of fibroblasts to myofibroblasts, which further worsens fibrosis. Although TGF-β has been extensively investigated in fibrosis, the roles of several components of its signaling pathway are still unknown. Endoglin is a co-receptor for TGF-β and is known to modulate TGF-β signaling. Therefore, endoglin could enhance the effects of TGF-β in fibrosis or act as an inhibitor. Multiple studies have been conducted that support either hypothesis. Elucidating the exact role of endoglin in TGF-β signaling during fibrosis is important in understanding the process of fibrosis and could lead to the development of better treatment.