The Role of Hippo/YAP Signaling in Alveolar Repair and Pulmonary Fibrosis.
The Role of Hippo/YAP Signaling in Alveolar Repair and Pulmonary Fibrosis.
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Hippo/YAP信号在肺泡修复和肺纤维化中的作用。
DOI：
10.3389/fmed.2021.752316
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发表时间：
2021
影响因子：
3.9
通讯作者：
Kropski JA
中科院分区：
文献类型：
--
作者：
Gokey JJ;Patel SD;Kropski JA
肺纤维化的特征是正常肺泡的丧失，病理性激活的成纤维细胞的堆积，以及大量的细胞外基质沉积，随着时间的推移，可导致呼吸功能的进行性丧失和死亡。这种呼吸功能的丧失与肺泡1型细胞(AT1)的丧失和肺泡2型细胞(AT2)的枯竭有关，前者在气体交换中起关键作用，后者在修复过程中作为祖细胞再生AT1和AT2细胞群。了解调节正常肺泡修复的机制和与病理修复相关的机制对于确定治疗或延缓纤维化疾病进展的潜在治疗靶点至关重要。河马/YAP发育信号通路被认为是正常肺泡发育和修复的调节因子。在特发性肺纤维化中，YAP/TAZ在肺泡上皮细胞和活化的成纤维细胞中的异常激活与纤维重塑的增加有关，这一途径作为抗纤维化治疗的靶点而引起人们的兴趣。在这篇综述中，我们总结了有关Hippo-YAP/TAZ通路在肺泡发育、动态平衡和修复中的作用的现有证据，并强调了必须解决的关键问题，以确定有效的策略来调节YAP/TAZ信号以防止进行性肺纤维化和增强适应性肺泡修复。
Pulmonary fibrosis is characterized by loss of normal alveoli, accumulation of pathologic activated fibroblasts, and exuberant extracellular matrix deposition that over time can lead to progressive loss of respiratory function and death. This loss of respiratory function is associated with the loss of alveolar type 1 cells (AT1) that play a crucial role in gas exchange and the depletion of the alveolar type 2 cells (AT2) that act as progenitor cells to regenerate the AT1 and AT2 cell populations during repair. Understanding the mechanisms that regulate normal alveolar repair and those associated with pathologic repair is essential to identify potential therapeutic targets to treat or delay progression of fibrotic diseases. The Hippo/YAP developmental signaling pathway has been implicated as a regulator of normal alveolar development and repair. In idiopathic pulmonary fibrosis, aberrant activation of YAP/TAZ has been demonstrated in both the alveolar epithelium and activated fibroblasts associated with increased fibrotic remodeling, and there is emerging interest in this pathway as a target for antifibrotic therapies. In this review, we summarize current evidence as to the role of the Hippo-YAP/TAZ pathway in alveolar development, homeostasis, and repair, and highlight key questions that must be resolved to determine effective strategies to modulate YAP/TAZ signaling to prevent progressive pulmonary fibrosis and enhance adaptive alveolar repair.
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影响因子：
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作者：
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通讯作者：
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