Abnormalities in cell polarity in polycystic kidney disease

我们检测了细胞粘附分子和间充质标志物的表达，以阐明PCK大鼠中细胞极性的丧失，PCK大鼠是一种在人类PKHDJ直向同源物中具有突变的ARPKD模型。在PCK大鼠中，根据囊肿扩大，囊小管中的E-钙粘蛋白和β-连环蛋白表达水平减弱并定位于外侧细胞-细胞接触。在这些肾单位的大囊肿中，尤其是邻近纤维化区的上皮细胞呈波形蛋白和纤维连接蛋白阳性。囊肿周围纤维化区波形蛋白和纤维连接蛋白阳性细胞增多。结论：EMT参与了PKD的病理生理过程。

We examined the expression of cell adhesion molecules and mesenchymal markers to clarify a loss of cell polarity in PCK rat, an ARPKD model with a mutation in the orthologue of human PKHDJ. E-cadherin and β-catenin expression levels in cystic tubule was attenuated and localized to lateral cell-cell contacts according to cyst enlargement in PCK rats. Some epithelial cells in large cysts derived from these segments of nephron, especially in adjacent fibrotic area, showed positive immunoreactivity of vimentin and fibronectin. Vimentin and fibronectin positive cells are increased in fibrotic area around cyst. In conclusions, these findings suggested that EMT is involved in pathophysiology of PKD.