Article
Hydrophobic pore gates regulate ion permeation in polycystic kidney disease 2 and 2L1 channels.
Nature communications - 13 Jun 2018
Zheng Wang, Yang Xiaoyong, Hu Ruikun, Cai Ruiqi, Hofmann Laura, Wang Zhifei, Hu Qiaolin, Liu Xiong, Bulkley David, Yu Yong, Tang Jingfeng, Flockerzi Veit, Cao Ying, Cao Erhu, Chen Xing-Zhen
Abstract excerpt
PKD2 and PKD1 genes are mutated in human autosomal dominant polycystic kidney disease. PKD2 can form either a homomeric cation channel or a heteromeric complex with the PKD1 receptor, presumed to respond to ligand(s) and/or mechanical stimuli. Here, we identify a two-residue hydrophobic gate in PKD2L1, and a single-residue hydrophobic gate in PKD2. We find that a PKD2 gain-of-function gate mutant effectively...
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