A mechanistic approach to inherited polycystic kidney disease

被引：0
作者：
John J. Bissler
Bradley P. Dixon
机构：
[1] Cincinnati Children’s Hospital Medical Center,Division of Nephrology and Hypertension
[2] Children’s Hospital Research Foundation,undefined
来源：
Pediatric Nephrology | 2005年 / 20卷
关键词：
Polycystic kidney disease; Nephronophthisis; Medullary cystic kidney; Tuberous sclerosis complex; Bardet-Biedl syndrome; Cilia;
D O I：
暂无
中图分类号：
学科分类号：
摘要：
There are approximately six and a half million people, of the estimated world population of six billion, with inherited polycystic kidney disease. Polycystic kidney diseases have a broad spectrum of associated findings that distinguish and define them as specific disease states. The dysregulation of renal tubular epithelial cell biology, including cell polarity, cell signaling, proliferation and apoptosis, basement membrane and matrix abnormalities, and fluid transport, has been postulated to contribute to cystogenesis. Evidence is currently accumulating that supports an association of the primary cilium and basal body, as well as the focal adhesion assembly, with polycystic kidney diseases. Renal cystogenesis may be the result of a disruption of a critical feedback loop that regulates tissue morphology based on the epithelial cell environment.
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页码：558 / 566
页数：8
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