Blocking TGFβ via Inhibition of the αvβ6 Integrin: A Possible Therapy for Systemic Sclerosis Interstitial Lung Disease.
Blocking TGFβ via Inhibition of the αvβ6 Integrin: A Possible Therapy for Systemic Sclerosis Interstitial Lung Disease.
复制标题
DOI：
10.1155/2011/208219
复制
发表时间：
2011
影响因子：
2.3
通讯作者：
Sheppard D
中科院分区：
文献类型：
--
作者：
Katsumoto TR;Violette SM;Sheppard D
间质性肺病（ILD）是系统性硬化症（SSc）的常见并发症，占SSc相关发病率和死亡率的很大比例。其发病机制仍然知之甚少，治疗SSc ILD的疗法充其量是次优的。SSc ILD发病机制可能与其他纤维化肺病有一些共同的机制，其中肺上皮细胞的失调可通过成纤维细胞的募集或原位生成和活化导致病理性纤维化。TGFβ是纤维化的主要调节因子，在肺中受整合素αvβ6的严格调节，整合素α vβ6在健康肺泡上皮细胞上以低水平表达，但在肺损伤或纤维化的情况下高度诱导。在这里，我们讨论了αvβ6的生物学，并将这种整合素作为SSc ILD背景下潜在的有吸引力的抑制靶点。
Interstitial lung disease (ILD) is a commonly encountered complication of systemic sclerosis (SSc) and accounts for a significant proportion of SSc-associated morbidity and mortality. Its pathogenesis remains poorly understood, and therapies that treat SSc ILD are suboptimal, at best. SSc ILD pathogenesis may share some common mechanisms with other fibrotic lung diseases, in which dysregulation of lung epithelium can contribute to pathologic fibrosis via recruitment or in situ generation and activation of fibroblasts. TGFβ, a master regulator of fibrosis, is tightly regulated in the lung by the integrin αvβ6, which is expressed at low levels on healthy alveolar epithelial cells but is highly induced in the setting of lung injury or fibrosis. Here we discuss the biology of αvβ6 and present this integrin as a potentially attractive target for inhibition in the setting of SSc ILD.