小窝蛋白-1与特发性肺纤维化

Caveolin-1 and odiopathic lung fibrosis

小窝蛋白作为小窝膜内的支架蛋白,能够组织和浓缩特异性的脂质,修饰信号转导分子以及负性调控许多信号转导通路和生物过程。生物体内小窝蛋白表达异常可引起疾病,特发性肺纤维化就是其中一种。大量研究表明,小窝蛋白-1在特发性肺纤维化发病过程中表达明显下调,大大降低了其对多条信号传导通路如TGF-β/SMAD、ERK/MAPK、JNK/MAPK和PKC等的负性调控作用,进而导致肺泡上皮细胞损伤、成纤维细胞增殖及表型转化、细胞外基质(ECM)重塑,加速了肺纤维化发病进程。因此该文就小窝蛋白-1在特发性肺纤维化中的作用进行了综述。

Caveolins are major structural proteins of eaveolae, which play important roles in organizing and concentrating specific lipids, modulating signals and regulating negatively various signaling pathways including TGF-β/SMAD,ERK/MAPK,JNK/ MAPK and PKC and many fundamental biologic processes. Abnormal expressions of caveolins have been involved in pathogenesis of many human diseases, such as idiopathetic lung fibrosis. As reported,low levels of caveolin-1 in patients with idiopathic lung fibrosis weakens its ability of regulating negatively signal transduction pathways, which leads to alveolar epithelial cells abnormal injury, lung fibroblasts proliferation and phenotype differentiation, and extracellular matrix remodeling resulting in development and progression of IPF.