Glomerulocystic kidney disease in mice with a targeted inactivation of Wwtr1.

ABSTRACT: Wwtr1 is a widely expressed 14-3-3-binding protein that regulates the activity of several transcription factors involved in development and disease. To elucidate the physiological role of Wwtr1, we generated Wwtr1-/- mice by homologous recombination. Surprisingly, although Wwtr1 is known to regulate the activity of Cbfa1, a transcription factor important for bone development, Wwtr1-/- mice show only minor skeletal defects. However, Wwtr1-/- animals present with renal cysts that lead to end-stage renal disease. Cysts predominantly originate from the dilation of Bowman's spaces and atrophy of glomerular tufts, reminiscent of glomerulocystic kidney disease in humans. A smaller fraction of cysts is derived from tubules, in particular the collecting duct (CD). The corticomedullary accumulation

SUBMITTER: Hossain Z 

PROVIDER: S-EPMC1785239 | biostudies-literature | 2007 Jan

REPOSITORIES: biostudies-literature