Latest progress on the molecular mechanisms of idiopathic pulmonary fibrosis
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Latest progress on the molecular mechanisms of idiopathic pulmonary fibrosis Yue Fang1,2 · Jingya Tian1,3 · Yumei Fan1 · Pengxiu Cao1 Received: 26 June 2020 / Accepted: 11 November 2020 © Springer Nature B.V. 2020
Abstract Idiopathic pulmonary fibrosis (IPF) is a serious life-threatening lung disease, and the median survival period of PF patients after diagnosis is only 2.5–3.5 years. At present, there are no effective drugs or therapeutics to reverse or even inhibit IPF. The main pathological characteristics of pulmonary fibrosis (PF) include damage to alveolar epithelial cells, fibroblast activation and extracellular matrix accumulation, which gradually lead to damage to the lung structure and decreased lung function. It is important to understand the cellular and molecular mechanisms of PF comprehensively and clearly. In this paper, critical signaling pathways related to PF were reviewed to present updates on the molecular mechanisms of PF. Keywords Idiopathic pulmonary fibrosis · Alveolar epithelial cells · Lung fibroblasts · Wnt · Mitochondria
Introduction Interstitial lung diseases (ILDs) are a group of heterogeneous pulmonary diseases characterized by diffuse pulmonary parenchyma, alveolar inflammation and interstitial fibrosis caused by a variety of environmental injuries, systemic diseases, and idiopathic diseases. Among ILDs, idiopathic pulmonary fibrosis (IPF) is the most common and has unknown etiology and poor prognosis [1]. It has been reported that the global mortality rate of IPF patients has been increasing annually. Among IPF patients, the mortality rate of men is higher than that of women, which may be related to Yue Fang and Jingya Tian have contributed to this work equally and ranked alphabetically. * Yumei Fan [email protected] * Pengxiu Cao [email protected] 1
Key Laboratory of Animal Physiology, Biochemistry and Molecular Biology of Hebei Province, College of Life Sciences, Hebei Normal University, 20 East Road of 2nd South Ring, Yuhua District, Shijiazhuang 050024, China
2
Key Laboratory of Brain Functional Genomics of Ministry of Education, School of Life Sciences, East China Normal University, Shanghai 200062, China
3
College of Chemistry and Environmental Sciences, Hebei University, Baoding, China
smoking, and the mortality rates of women and nonsmokers are lower than those of men and nonsmokers [2]. In PF, collagen deposition in the lung parenchyma is associated with the accumulation of fibroblasts, which are located below the epithelial surface. The epithelia surface is composed of proliferating type II alveolar epithelial cells (AECIIs) or epithelium with a bronchiolar appearance. Environmentally induced damage to susceptible AECs leads to increased cell death or epithelial damage, and the pathological interactions of AECs with fibroblasts result in activated fibroblasts that produce excessive collagen and other extracellular matrix [3]. In addition to AECs and fibroblasts, inflammatory cells are also involved in the pathogenesis of this disease by
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