Zhou Jing
Renal Division, Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts 02115, USA.
Annu Rev Physiol. 2009;71:83-113. doi: 10.1146/annurev.physiol.70.113006.100621.
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.
多囊蛋白是一类由序列同源性联系在一起的八次跨膜蛋白家族。该名称源于在多囊肾病中编码多囊蛋白-1和-2的基因突变的鉴定。本综述讨论了多囊蛋白研究中的最新话题，重点是多囊蛋白-1和多囊蛋白-2在初级纤毛和细胞周期中的作用。多囊蛋白在发育过程中似乎起着关键作用，但一个主要问题是它们在成熟器官中的功能。研究了它们在初级纤毛、剪切力感知、细胞内钙改变和平面细胞极性方面的作用。还讨论了多囊肾病的第三次打击假说。
