Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung 80756, Taiwan.
Department of Pediatrics, School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung 80708, Taiwan.
Int J Mol Sci. 2023 Jun 29;24(13):10876. doi: 10.3390/ijms241310876.
Pulmonary fibrosis (PF) is a chronic lung disorder characterized by the presence of scarred and thickened lung tissues. Although the Food and Drug Administration approved two antifibrotic drugs, pirfenidone, and nintedanib, that are currently utilized for treating idiopathic PF (IPF), the clinical therapeutic efficacy remains unsatisfactory. It is crucial to develop new drugs or treatment schemes that combine pirfenidone or nintedanib to achieve more effective outcomes for PF patients. Understanding the complex mechanisms underlying PF could potentially facilitate drug discovery. Previous studies have found that the activation of inflammasomes, including nucleotide-binding and oligomerization domain (NOD)-like receptor protein (NLRP)1, NLRP3, NOD-like receptor C4, and absent in melanoma (AIM)2, contributes to lung inflammation and fibrosis. This article aims to summarize the cellular and molecular regulatory cues that contribute to PF with a particular emphasis on the role of AIM2 inflammasome in mediating pathophysiologic events during PF development. The insights gained from this research may pave the way for the development of more effective strategies for the prevention and treatment of PF.
肺纤维化 (PF) 是一种慢性肺部疾病,其特征是存在疤痕和增厚的肺组织。尽管美国食品和药物管理局批准了两种抗纤维化药物,吡非尼酮和尼达尼布,目前用于治疗特发性肺纤维化 (IPF),但临床治疗效果仍不尽如人意。开发新的药物或治疗方案,结合吡非尼酮或尼达尼布,以实现更有效的肺纤维化患者的治疗效果至关重要。了解肺纤维化的复杂机制可能有助于药物发现。先前的研究发现,炎症小体的激活,包括核苷酸结合和寡聚结构域(NOD)样受体蛋白(NLRP)1、NLRP3、NOD 样受体 C4 和黑色素瘤缺失(AIM)2,有助于肺炎症和纤维化。本文旨在总结导致 PF 的细胞和分子调节线索,特别强调 AIM2 炎症小体在介导 PF 发展过程中的病理生理事件中的作用。这项研究的结果可能为预防和治疗 PF 的更有效策略的发展铺平道路。