CXCL12/CXCR4生物轴与特发性肺纤维化研究进展

Research on CXCL12/CXCR4 biological axis and idiopathic pulmonary fibrosis

特发性肺纤维化(idiopathic pulmonary fibrosis,IPF)是一种病因未明的肺间质性疾病,以肺泡上皮损伤、纤维细胞增殖、肌成纤维细胞形成、细胞外基质沉积为主要病理特征。CXCR4是纤维细胞主要的趋化因子受体,CXCL12是CXCR4的唯一配体。大量研究表明,在缺氧、HIF-1α、PI3K-Akt-mTOR路径的调节下,CXCL12/CXCR4生物轴(CXCL12/CX-CR4biological axis)能促进纤维细胞增殖、肌成纤维细胞形成、细胞外基质沉积,加速肺纤维化发病进程。该文将就CXCL12/CXCR4生物轴在特发性肺纤维化发病过程中的作用进行综述。

Idiopathic pulmonary fibrosis（IPF）,with unknown pathogeny,is an interstitial lung disease.The pathological features are diffuse epithelial-cell lesion,fibroblast proliferation,myofibroblast differentiation and excessive extracellular matrix deposition.CXCR4 is the predominant chemokine receptor on fibrocytes;CXCL12 is the only ligand of CXCR4.A large number of studies have shown that CXCL12/CXCR4 biological axis plays an important role in the pathogenesis of idiopathic pulmonary fibrosis.Under the regulation of hypoxia,HIF-1α and PI3K-Akt-mTOR path,CXCL12/CXCR4 biological axis promotes lung fibroblast proliferation,myofibroblast differentiation and extracellular matrix deposition,resulting in development and progression of IPF.