哺乳动物呼吸系统或肺是一种像树一样的分枝结构，也是与外部环境进行气体交换的主要场所。在结构上，肺被广泛分为近端（或传导）气道和远端肺泡区域，气体交换发生于此处。与呼吸树并行，肺血管起始于大肺动脉，迅速分支，最终在肺泡结构相邻的毛细血管处结束，以实现气体交换。NOTCH信号通路在肺的发育、分化和受伤后的再生中起着重要作用。通过活化四个NOTCH受体（NOTCH1-4）传递NOTCH信号通路的信号介导每个受体调控独特的生物过程。NOTCH信号通路的失调已与多种成人急性和慢性肺疾病的发育和病理生理学相关联。其中包括越来越多的证据表明，NOTCH3信号通路的改变在慢性阻塞性肺疾病、肺癌、哮喘、特发性肺纤维化和肺动脉高压的发展和病理发生中起着重要作用。在此，我们提供了NOTCH3信号通路在调节成人肺的修复/再生、与肺疾病发展的关联以及针对其信号活性的潜在治疗策略的全面总结。

The mammalian respiratory system or lung is a tree-like branching structure, and the main site of gas exchange with the external environment. Structurally, the lung is broadly classified into the proximal (or conducting) airways and the distal alveolar region, where the gas exchange occurs. In parallel with the respiratory tree, the pulmonary vasculature starts with large pulmonary arteries that subdivide rapidly ending in capillaries adjacent to alveolar structures to enable gas exchange. The NOTCH signalling pathway plays an important role in lung development, differentiation and regeneration post-injury. Signalling via the NOTCH pathway is mediated through activation of four NOTCH receptors (NOTCH1-4), with each receptor capable of regulating unique biological processes. Dysregulation of the NOTCH pathway has been associated with development and pathophysiology of multiple adult acute and chronic lung diseases. This includes accumulating evidence that alteration of NOTCH3 signalling plays an important role in the development and pathogenesis of chronic obstructive pulmonary disease, lung cancer, asthma, idiopathic pulmonary fibrosis and pulmonary arterial hypertension. Herein, we provide a comprehensive summary of the role of NOTCH3 signalling in regulating repair/regeneration of the adult lung, its association with development of lung disease and potential therapeutic strategies to target its signalling activity.