Liu Xiong, Tang Jingfeng, Chen Xing-Zhen
Membrane Protein Disease Research Group, Department of Physiology, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, AB, Canada.
National "111" Center for Cellular Regulation and Molecular Pharmaceutics, Hubei University of Technology, Wuhan, HB, China.
Front Physiol. 2022 Aug 10;13:962571. doi: 10.3389/fphys.2022.962571. eCollection 2022.
Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in the PKD1 or PKD2 gene which encodes membrane receptor PKD1 and cation channel PKD2, respectively. PKD2, also called transient receptor potential polycystin-2 (TRPP2), is a Ca-permeable channel located on the membrane of cell surface, primary cilia, and endoplasmic reticulum (ER). Ca is closely associated with diverse cellular functions. While ER Ca homeostasis depends on different Ca receptors, channels and transporters, the role of PKD2 within the ER remains controversial. Whether and how PKD2-mediated ER Ca leak relates to ADPKD pathogenesis is not well understood. Here, we reviewed current knowledge about the biophysical and physiological properties of PKD2 and how PKD2 contributes to ER Ca homeostasis.
常染色体显性多囊肾病（ADPKD）是由PKD1或PKD2基因突变引起的，这两个基因分别编码膜受体PKD1和阳离子通道PKD2。PKD2，也称为瞬时受体电位多囊蛋白-2（TRPP2），是一种位于细胞表面、初级纤毛和内质网（ER）膜上的钙通透通道。钙与多种细胞功能密切相关。虽然内质网钙稳态依赖于不同的钙受体、通道和转运蛋白，但PKD2在内质网中的作用仍存在争议。PKD2介导的内质网钙泄漏是否以及如何与ADPKD发病机制相关尚不清楚。在这里，我们综述了关于PKD2生物物理和生理特性的现有知识，以及PKD2如何促进内质网钙稳态。
