1 Department of Pharmacology.
Am J Respir Cell Mol Biol. 2013 Dec;49(6):912-22. doi: 10.1165/rcmb.2013-0070OC.
Idiopathic pulmonary fibrosis is a devastating disease characterized by alveolar epithelial cell injury, the accumulation of fibroblasts/myofibroblasts, and the deposition of extracellular matrix proteins. Lysophosphatidic acid (LPA) signaling through its G protein-coupled receptors is critical for its various biological functions. Recently, LPA and LPA receptor 1 were implicated in lung fibrogenesis. However, the role of other LPA receptors in fibrosis remains unclear. Here, we use a bleomycin-induced pulmonary fibrosis model to investigate the roles of LPA2 in pulmonary fibrogenesis. In the present study, we found that LPA2 knockout (Lpar2(-/-)) mice were protected against bleomycin-induced lung injury, fibrosis, and mortality, compared with wild-type control mice. Furthermore, LPA2 deficiency attenuated the bleomycin-induced expression of fibronectin (FN), α-smooth muscle actin (α-SMA), and collagen in lung tissue, as well as levels of IL-6, transforming growth factor-β (TGF-β), and total protein in bronchoalveolar lavage fluid. In human lung fibroblasts, the knockdown of LPA2 attenuated the LPA-induced expression of TGF-β1 and the differentiation of lung fibroblasts to myofibroblasts, resulting in the decreased expression of FN, α-SMA, and collagen, as well as decreased activation of extracellular regulated kinase 1/2, Akt, Smad3, and p38 mitogen-activated protein kinase. Moreover, the knockdown of LPA2 with small interfering RNA also mitigated the TGF-β1-induced differentiation of lung fibroblasts. In addition, LPA2 deficiency significantly attenuated the bleomycin-induced apoptosis of alveolar and bronchial epithelial cells in the mouse lung. Together, our data indicate that the knockdown of LPA2 attenuated bleomycin-induced lung injury and pulmonary fibrosis, and this may be related to an inhibition of the LPA-induced expression of TGF-β and the activation and differentiation of fibroblasts.
特发性肺纤维化是一种破坏性疾病,其特征在于肺泡上皮细胞损伤、成纤维细胞/肌成纤维细胞的积累以及细胞外基质蛋白的沉积。溶血磷脂酸(LPA)通过其 G 蛋白偶联受体信号传导对于其各种生物学功能至关重要。最近,LPA 和 LPA 受体 1 被牵连到肺纤维化的发生中。然而,其他 LPA 受体在纤维化中的作用尚不清楚。在这里,我们使用博来霉素诱导的肺纤维化模型来研究 LPA2 在肺纤维化中的作用。在本研究中,我们发现与野生型对照小鼠相比,LPA2 敲除(Lpar2(-/-))小鼠对博来霉素诱导的肺损伤、纤维化和死亡率具有保护作用。此外,LPA2 缺乏减弱了博来霉素诱导的肺组织中纤维连接蛋白(FN)、α-平滑肌肌动蛋白(α-SMA)和胶原的表达,以及支气管肺泡灌洗液中白细胞介素-6(IL-6)、转化生长因子-β(TGF-β)和总蛋白的水平。在人肺成纤维细胞中,LPA2 的敲低减弱了 LPA 诱导的 TGF-β1 表达和肺成纤维细胞向肌成纤维细胞的分化,导致 FN、α-SMA 和胶原的表达减少,以及细胞外调节激酶 1/2、Akt、Smad3 和 p38 丝裂原活化蛋白激酶的活性降低。此外,用小干扰 RNA 敲低 LPA2 也减轻了 TGF-β1 诱导的肺成纤维细胞分化。此外,LPA2 缺乏显著减弱了博来霉素诱导的小鼠肺中肺泡和支气管上皮细胞的凋亡。总之,我们的数据表明,LPA2 的敲低减弱了博来霉素诱导的肺损伤和肺纤维化,这可能与抑制 LPA 诱导的 TGF-β表达以及成纤维细胞的激活和分化有关。