Article
Impaired formation of desmosomal junctions in ADPKD epithelia.
Histochemistry and cell biology - 1 Dec 2005
Russo Ryan J, Husson Hervé, Joly Dominique, Bukanov Nikolay O, Patey Natacha, Knebelmann Bertrand, Ibraghimov-Beskrovnaya Oxana
Abstract excerpt
Mutations in polycystin-1 (PC-1) are responsible for autosomal dominant polycystic kidney disease (ADPKD), characterized by formation of fluid-filled tubular cysts. The PC-1 is a multifunctional protein essential for tubular differentiation and maturation found in desmosomal junctions of epithelial cells where its primary function is to mediate cell-cell adhesion. To address the impact of mutated PC-1 on...
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