Hydrophobic pore gates regulate ion permeation in polycystic kidney disease 2 and 2L1 channels.
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ABSTRACT:
SUBMITTER: Zheng W
PROVIDER: S-EPMC5998024 | biostudies-literature | 2018 Jun
REPOSITORIES: biostudies-literature
Publications
Hydrophobic pore gates regulate ion permeation in polycystic kidney disease 2 and 2L1 channels.
Zheng Wang W Yang Xiaoyong X Hu Ruikun R Cai Ruiqi R Hofmann Laura L Wang Zhifei Z Hu Qiaolin Q Liu Xiong X Bulkley David D Yu Yong Y Tang Jingfeng J Flockerzi Veit V Cao Ying Y Cao Erhu E Chen Xing-Zhen XZ
Nature communications 20180613 1
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 rescues PKD2 knockdown-induced phenotypes in embryonic zebrafish. The structure of a PKD ...[more]
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