肺血管重建（PVR）是肝肺综合征（HPS）等疾病的重要病理生理基础。前期研究发现肺动脉平滑肌细胞（PASMCs）骨架重塑可能是其表型转换及增殖的上游关键事件。既往报道，整合素β1内化再循环调控细胞骨架重塑；这一过程受磷酸化ICAP-1调控，而ICAP-1磷酸化活性可能受Decorin调节。预实验发现，HPS大鼠肺微血管整合素β1表达上调；HPS血清刺激下PASMCs中Decorin表达下调。由此推测，Decorin-ICAP-1调控的整合素β1内化再循环，进而调节PASMCs骨架重塑可能是PVR的重要分子机制。课题拟建立HPS模型，采用WB、qPCR、激光共聚焦等方法，观察细胞骨架重塑；通过俘获酶联免疫蛋白示踪、流式细胞等技术，检测整合素β1内化再循环；采用慢病毒及siRNA干预Decorin表达，检测其对整合素β1内化再循环的调控效应；研究结果可能为HPS等疾病的防治提供新靶点。

Pulmonary vascular remodeling (PVR) is the important pathophysiological foundation of hepatopulmonary syndrome (HPS) and other diseases. Our previous research have found that pulmonary artery smooth muscle cells (PASMCs) occurred cytoskeleton remodeling, which as a key upstream event before cells phenotype modulation and abnormal proliferation may play an important role in the PVR. It has been reported that the internalization and recycling of integrinβ1 could regulate the cells cytoskeleton remodeling; which was regulated by p-ICAP-1. And the phosphorylation activity of ICAP-1 was regulated by Decorin. Preliminary experiments found the up-regulating of integrinβ1 expression in HPS rat lung tissue, while HPS rat serum suppressed the protein expression level of Decorin in PASMCs. According to the results above, we hypothesised that the internalization and recycling of integrinβ1 by Decorin-ICAP-1 leads to cells cytoskeleton remodeling, which is the molecular mechanisms of PVR. This study intends to establish HPS rats models in vivo and in vitro, using WB, qPCR, Laser scanning confocal and other methods to observe PASMCs cytoskeleton remodeling; Capture-ELISA and flow cytometry techniques to detect the internalization and recycling of integrinβ1 ; using transfection of specific decorin lentivirus and siRNA to regulate the expression of decorin, by which to research the regulatory role of the decorin mediated the internalization and recycling of integrinβ1. The results of this study may provide a new target for the prevention and treatment of diseases such as HPS.