Zabihi Mahsa, Shahriari Felordi Mahtab, Lingampally Arun, Bellusci Saverio, Chu Xuran, El Agha Elie
Department of Medicine V, Internal Medicine, Infectious Diseases and Infection Control, Universities of Giessen and Marburg Lung Center (UGMLC), German Center for Lung Research (DZL), Justus-Liebig University Giessen, Giessen 35392, Germany.
Cardio-Pulmonary Institute (CPI), Giessen 35392, Germany.
Chin Med J Pulm Crit Care Med. 2024 Sep 17;2(3):142-150. doi: 10.1016/j.pccm.2024.08.003. eCollection 2024 Sep.
Idiopathic pulmonary fibrosis (IPF) is characterized by accumulation of myofibroblasts (MYFs) and extracellular matrix components, which leads to severe distortion and scarring of the gas exchange units of the lung, the alveoli, and ultimately respiratory failure. Fibrosis-associated MYFs are therefore widely regarded as the culprits that compromise the architectural makeup of the lung in fibrotic disease. During the past decade, the cellular source of MYFs has been intensely investigated. The rationale for such studies is that identifying the origin of these cells might help identify novel therapeutic targets and candidates to treat IPF patients. Recent advances in basic and translational research employing lineage tracing and multi-omics approaches have helped address the identity of MYF precursors, highlight the underlying heterogeneity, and to a less extent investigate MYF fate during fibrosis resolution. In this review, we discuss the current understanding of such important aspects of MYF biology as well as recent developments in the treatment of IPF.
特发性肺纤维化（IPF）的特征是肌成纤维细胞（MYFs）和细胞外基质成分的积累，这会导致肺部气体交换单位（肺泡）严重变形和瘢痕形成，最终导致呼吸衰竭。因此，纤维化相关的肌成纤维细胞被广泛认为是导致纤维化疾病中肺结构组成受损的罪魁祸首。在过去十年中，人们对肌成纤维细胞的细胞来源进行了深入研究。此类研究的基本原理是，确定这些细胞的起源可能有助于识别治疗IPF患者的新治疗靶点和候选药物。采用谱系追踪和多组学方法的基础研究和转化研究的最新进展，有助于明确肌成纤维细胞前体的身份，突出其潜在的异质性，并在较小程度上研究纤维化消退过程中肌成纤维细胞的命运。在这篇综述中，我们讨论了目前对肌成纤维细胞生物学这些重要方面的理解以及IPF治疗的最新进展。
