纤毛
多囊肾病
生物
细胞生物学
包装D1
跨膜蛋白
遗传学
肾
受体
出处
期刊:Annual Review of Physiology
[Annual Reviews]
日期:2009-03-01
卷期号:71 (1): 83-113
被引量:183
标识
DOI:10.1146/annurev.physiol.70.113006.100621
摘要
Polycystins are a family of eight-transmembrane proteins united by sequence homology. The name stems from the identification of mutations in genes encoding polycystin-1 and -2 in polycystic kidney diseases. This review discusses recent topics in polycystin research, with a focus on the role of polycystin-1 and polycystin-2 in primary cilia and the cell cycle. Polycystins appear to play key roles during development, but a major question is their function in mature organs. Their roles in primary cilia, shear stress sensation, alteration of intracellular calcium, and planar cell polarity are examined. The third-hit hypothesis of polycystic kidney disease is discussed.
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