Toxic tubular injury in kidneys from Pkd1-deletion mice accelerates cystogenesis accompanied by dysregulated planar cell polarity and canonical Wnt signaling pathways

• Published in: Human molecular genetics Vol. 18; no. 14; pp. 2532 - 2542
• Main Authors: Happé, Hester, Leonhard, Wouter N., van der Wal, Annemieke, van de Water, Bob, Lantinga-van Leeuwen, Irma S., Breuning, Martijn H., de Heer, Emile, Peters, Dorien J.M.
• Format: Journal Article
• Language: English
• Published: Oxford Oxford University Press 15.07.2009; Oxford Publishing Limited (England)
• Subjects: Animals; Biological and medical sciences; Cell physiology; Cell Polarity; Cell Proliferation; Disease Models, Animal; Fundamental and applied biological sciences. Psychology; Gene Deletion; Genetics of eukaryotes. Biological and molecular evolution; Humans; Kidney Tubules - cytology; Kidney Tubules - injuries; Kidney Tubules - metabolism; Male; Mice; Mice, Knockout; Molecular and cellular biology; Nerve Tissue Proteins - genetics; Nerve Tissue Proteins - metabolism; Polycystic Kidney, Autosomal Dominant - genetics; Polycystic Kidney, Autosomal Dominant - metabolism; Polycystic Kidney, Autosomal Dominant - physiopathology; Signal Transduction; TRPP Cation Channels - genetics; TRPP Cation Channels - metabolism; Wnt Proteins - genetics; Wnt Proteins - metabolism; Chromosomal aberration; Signal transduction; Vertebrata; Mammalia; Urinary system; Mouse; Rodentia; Deletion; Genetics; Cell polarity; Kidney

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by large fluid-filled cysts and progressive deterioration of renal function necessitating renal replacement therapy. Previously, we generated a tamoxifen-inducible, kidney epithelium-specific Pkd1-deletion mouse model and showed t...