Fibronectin and its role in polycystic kidney diseases (B03)

该项目将调查细胞外基质成分糖蛋白纤维连接蛋白是否在预防多囊肾病(PKD)中发挥重要作用。这一假说基于的初步发现表明，新生小鼠体内纤维连接蛋白的诱导缺失会导致皮髓质肾囊肿的形成，并导致肾脏的完整性和功能丧失。我们将描述在小鼠纤维连接蛋白缺失后导致囊变形成的分子机制，并期待这些发现将有助于我们理解人类PKD背后的分子致病事件。

The project will investigate the question if the glycoprotein fibronectin, an extracellular matrix constituent, plays an essential role for the prevention of polycystic kidney disease (PKD). This hypothesis is based on preliminary findings indicating that the induced deletion of fibronectin in newborn mice results in the formation of corticomedullary kidney cysts and the loss of kidney integrity and function. We will characterize the molecular mechanisms that lead to cyst formation following fibronectin deletion in the mouse and expect that the findings will contribute to our understanding of the molecular pathogenetic events that are behind PKD in humans.