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Published on: June 6, 2017
Polycystins and primary cilia: primers for cell cycle progression
1Renal Division, Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts 02115, USA. zhou@rics.bwh.harvard.edu
Annual Review of Physiology
|July 4, 2009
Summary
Polycystin proteins are crucial for kidney development and function. This review explores their roles in primary cilia, cell cycle, and the third-hit hypothesis of polycystic kidney disease.
Area of Science:
- Molecular Biology
- Cell Biology
- Genetics
Background:
- Polycystins are integral membrane proteins associated with polycystic kidney diseases.
- Mutations in polycystin-1 and polycystin-2 genes are primary causes of these inherited renal disorders.
- Understanding polycystin function is key to addressing polycystic kidney disease pathogenesis.
Purpose of the Study:
- To review recent advancements in polycystin research.
- To elucidate the roles of polycystin-1 and polycystin-2 in primary cilia and cell cycle regulation.
- To examine polycystin involvement in mature organ function, shear stress sensing, calcium signaling, and planar cell polarity.
Main Methods:
- Literature review of recent polycystin research.
- Analysis of studies focusing on polycystin function in development and mature organs.
- Discussion of the third-hit hypothesis in the context of polycystic kidney disease.
Main Results:
- Polycystins play significant roles during embryonic development.
- Evidence suggests polycystins are involved in primary cilia function, mechanosensation, and intracellular calcium modulation.
- The third-hit hypothesis offers a framework for understanding polycystic kidney disease progression.
Conclusions:
- Polycystins are essential for normal development and organ function.
- Further research into polycystin roles in primary cilia and cell cycle control is warranted.
- Understanding polycystin biology may lead to novel therapeutic strategies for polycystic kidney disease.
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