细胞外基质
特发性肺纤维化
医学
纤维化
发病机制
肺纤维化
整合素
间质性肺病
肺
信号转导
癌症研究
免疫学
细胞生物学
病理
生物
受体
内科学
作者
Ioannis Tomos,Argyrios Tzouvelekis,Vassilis Aidinis,Effrosyni D. Manali,Evangelos Bouros,Demosthenes Bouros,Spyros Papiris
标识
DOI:10.1080/17476348.2017.1300533
摘要
Introduction: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease characterized by irreversible fibrosis. Current disease pathogenesis assumes an aberrant wound healing process in response to repetitive injurious stimuli leading to apoptosis of epithelial cells, activation of fibroblasts and accumulation of extracellular matrix (ECM). Particularly, lung ECM is a highly dynamic structure that lies at the core of several physiological and developmental pathways. The scope of this review article is to summarize current knowledge on the role of ECM in the pathogenesis of IPF, unravel novel mechanistic data and identify future more effective therapeutic targets.Areas covered: The exact mechanisms through which lung microenvironment activates fibroblasts and inflammatory cells, regulates profibrotic signaling cascades through growth factors, integrins and degradation enzymes ultimately leading to excessive matrix deposition are discussed. Furthermore, the potential therapeutic usefulness of specific inhibitors of matrix deposition or activators of matrix degradation pathways are also presented.Expert commentary: With a gradually increasing worldwide incidence IPF still present a major challenge in clinical research due to its unknown etiopathogenesis and current ineffective treatment approaches. Today, there is an amenable need for more effective therapeutic targets and ECM components may represent one.
科研通智能强力驱动
Strongly Powered by AbleSci AI