【摘要】 特发性肺纤维化(idiopathic pulmonary fibrosis, IPF)是一种病因复杂且缺乏有效治疗手段的严重慢性肺部疾病。该研究通过构建转基因小鼠模型,特异性地在肺泡Ⅱ型上皮细胞中过表达肺表面活性蛋白B(surfactant protein B, SP-B),探讨其在IPF中的功能及其作用机制。研究结果发现了SP-B的过表达会导致小鼠肺组织结构受损、细胞外基质异常沉积,并引发肺部炎症反应,揭示了SP-B在IPF病理进程中的重要作用。此外, SP-B可能通过调控TGF-β信号通路影响细胞增殖和细胞外基质蛋白表达,从而促进肺纤维化的发展。该研究为IPF的病因学研究提供了新的视角,并为基于Sftpb基因的治疗策略提供了潜在的靶点。更多还原

【Abstract】 IPF(idiopathic pulmonary fibrosis) is a severe chronic lung disease with complex etiology and lacking effective treatment. This study aimed to investigate the function and mechanism of pulmonary SP-B(surfactant protein B) in IPF by constructing a transgenic mouse model that specifically overexpressed SP-B in alveolar type Ⅱ epithelial cells. The overexpression of SP-B led to structural damage in lung tissue, abnormal extracellular matrix deposition, and pulmonary inflammation, highlighting its critical role in IPF pathogenesis. Furthermore,SP-B promoted pulmonary fibrosis by regulating the TGF-β signaling pathway to affect cell proliferation and extracellular matrix protein expression. These findings offer new insights into the etiology of IPF and potential targets for Sftpb gene-based therapeutic strategies.更多还原