Abnormalities in cell polarity in polycystic kidney disease

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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.