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HIF-1α promotes cyst progression in a mouse model of autosomal dominant polycystic kidney disease

Kraus, Andre ; Peters, Dorien J.M. ; Klanke, Bernd ; Weidemann, Alexander ; Willam, Carsten ; Schley, Gunnar ; Kunzelmann, Karl ; Eckardt, Kai-Uwe ; Buchholz, Bjoern



Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is mainly caused by mutations of the PKD1 gene and characterized by growth of bilateral renal cysts. Cyst growth is accompanied by regional hypoxia and induction of hypoxia-inducible factor (HIF)-1 alpha in cyst-lining epithelial cells. To determine the relevance of HIF-1 alpha for cyst growth in vivo we used an inducible kidney ...

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