Lagares David, Ghassemi-Kakroodi Parisa, Tremblay Caroline, Santos Alba, Probst Clemens K, Franklin Alicia, Santos Daniela M, Grasberger Paula, Ahluwalia Neil, Montesi Sydney B, Shea Barry S, Black Katharine E, Knipe Rachel, Blati Meryem, Baron Murray, Wu Brian, Fahmi Hassan, Gandhi Rajiv, Pardo Annie, Selman Moisés, Wu Jiangping, Pelletier Jean-Pierre, Martel-Pelletier Johanne, Tager Andrew M, Kapoor Mohit
Division of Pulmonary and Critical Care Medicine, Fibrosis Research Center and Center for Immunology and Inflammatory Diseases, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Department of Medicine, University of Montreal Hospital Research Centre (CRCHUM), Montreal, Québec, Canada.
Nat Med. 2017 Dec;23(12):1405-1415. doi: 10.1038/nm.4419. Epub 2017 Oct 23.
Maladaptive wound healing responses to chronic tissue injury result in organ fibrosis. Fibrosis, which entails excessive extracellular matrix (ECM) deposition and tissue remodeling by activated myofibroblasts, leads to loss of proper tissue architecture and organ function; however, the molecular mediators of myofibroblast activation have yet to be fully identified. Here we identify soluble ephrin-B2 (sEphrin-B2) as a new profibrotic mediator in lung and skin fibrosis. We provide molecular, functional and translational evidence that the ectodomain of membrane-bound ephrin-B2 is shed from fibroblasts into the alveolar airspace after lung injury. Shedding of sEphrin-B2 promotes fibroblast chemotaxis and activation via EphB3 and/or EphB4 receptor signaling. We found that mice lacking ephrin-B2 in fibroblasts are protected from skin and lung fibrosis and that a disintegrin and metalloproteinase 10 (ADAM10) is the major ephrin-B2 sheddase in fibroblasts. ADAM10 expression is increased by transforming growth factor (TGF)-β1, and ADAM10-mediated sEphrin-B2 generation is required for TGF-β1-induced myofibroblast activation. Pharmacological inhibition of ADAM10 reduces sEphrin-B2 levels in bronchoalveolar lavage and prevents lung fibrosis in mice. Consistent with the mouse data, ADAM10-sEphrin-B2 signaling is upregulated in fibroblasts from human subjects with idiopathic pulmonary fibrosis. These results uncover a new molecular mechanism of tissue fibrogenesis and identify sEphrin-B2, its receptors EphB3 and EphB4 and ADAM10 as potential therapeutic targets in the treatment of fibrotic diseases.
对慢性组织损伤的适应性伤口愈合反应会导致器官纤维化。纤维化涉及过多的细胞外基质(ECM)沉积以及激活的肌成纤维细胞进行的组织重塑,会导致正常组织结构和器官功能丧失;然而,肌成纤维细胞激活的分子介质尚未完全确定。在此,我们确定可溶性ephrin-B2(sEphrin-B2)是肺和皮肤纤维化中的一种新的促纤维化介质。我们提供了分子、功能和转化方面的证据,表明膜结合型ephrin-B2的胞外域在肺损伤后从成纤维细胞脱落到肺泡腔中。sEphrin-B2的脱落通过EphB3和/或EphB4受体信号促进成纤维细胞趋化性和激活。我们发现,成纤维细胞中缺乏ephrin-B2的小鼠可免受皮肤和肺纤维化的影响,并且解整合素和金属蛋白酶10(ADAM10)是成纤维细胞中主要的ephrin-B2裂解酶。转化生长因子(TGF)-β1可增加ADAM10的表达,TGF-β1诱导的肌成纤维细胞激活需要ADAM10介导的sEphrin-B2生成。对ADAM10的药理抑制可降低支气管肺泡灌洗中的sEphrin-B2水平,并预防小鼠肺纤维化。与小鼠数据一致,特发性肺纤维化患者的成纤维细胞中ADAM10-sEphrin-B2信号上调。这些结果揭示了组织纤维化形成的一种新分子机制,并确定sEphrin-B2、其受体EphB3和EphB4以及ADAM10是治疗纤维化疾病的潜在治疗靶点。