The role of primary cilia in lung development and diseases

This is the authors' abstract. We don't add key points for this paper.

The primary cilium is a solitary, non-motile, microtubule-based structure that projects from the surface of most mammalian cells. Once regarded as an evolutionary relic, the primary cilium is now recognized as a vital signaling hub coordinating key cellular processes including cell cycle progression, differentiation, and the maintenance of tissue homeostasis. In the lung, primary cilia exhibit dynamic spatiotemporal patterns: they are abundant during early lung development and show cell type- and context-dependent changes in mature tissues, with altered ciliogenesis observed during injury and repair. This review summarizes the dynamic functions of primary cilia in lung development and their associations with major signaling and sensory pathways, including Hedgehog, transforming growth factor-β/bone morphogenetic protein (TGF-β/BMP), Notch, Wnt, platelet-derived growth factor (PDGF) signaling, and mechanosensitive pathways. We also focus on the growing evidence linking primary ciliary dysfunction to major respiratory diseases including asthma, chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, pulmonary arterial hypertension and lung cancer. Understanding the biology of primary cilia not only provides critical insights into the development of respiratory diseases but also holds significant promise for identifying novel therapeutic targets.