何文早1,2,贾振华1,2,黄 渊1,2*,唐景峰1,2,陈兴珍1,2*
(1 湖北工业大学生物工程与食品学院,武汉 430068;2 湖北工业大学国家外专局/教育部细胞调控与分子药物“111”引智基地,武汉 430068)

摘 要:摘 要:PKD2L1 是常染色体显性多囊肾病(autosomal dominant polycystic kidney disease, ADPKD) 的致病基因PKD2 的同源基因,也称为TRPP3 (transient receptor potential cation channel, subfamily p, member 3)。PKD2L1是一个非选择性的阳离子通道,具有高度Ca2+ 渗透性,可与PKD1L3 形成异源四聚体,作为酸味受体进行酸信号的传递。该文系统地阐述了PKD2L1 的结构和其他调控蛋白对PKD2L1 通道活性的影响、PKD2L1的off-response 反应与酸信号的传递、PKD2L1 蛋白翻译后修饰,同时,也总结了PKD2L1 在心肌肥大以及维持脊柱形态中的作用。

Recent advances in PKD2L1 ion channel function and its protein modification
HE Wen-Zao1,2, JIA Zhen-Hua1,2, HUANG Yuan1,2*, TANG Jing-Feng1,2, CHEN Xing-Zhen1,2*
(1 School of Food and Biological Engineering, Hubei University of Technology, Wuhan 430068, China; 2 HBUT National "111" Center for Cellular Regulation and Molecular Pharmaceutics, Hubei University of Technology, Wuhan 430068, China)

Abstract: Abstract: PKD2L1 is a homologous gene of the PKD2, which is the pathogenic gene of the autosomal dominant polycystic kidney disease (ADPKD), also known as TRPP3 (transient receptor potential cation channel, subfamily p, member 3). PKD2L1 is a non-selective cation channel, which has high Ca2+ permeability and can form heterotetramers with PKD1L3 to transmit acid signals as a sour receptor. This paper systematically describes the structure of PKD2L1, the influence of other regulatory proteins on the activity of PKD2L1 channel, the off-response reaction of PKD2L1 and the acid signal transmission, the post-translational modification of PKD2L1 protein. It also summarizes the role of PKD2L1 in myocardial hypertrophy and maintenance of spinal morphology.

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