Architecture and functional properties of the CFTR channel pore

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• 作者：Paul Linsdell
• 关键词：ABC protein ; CFTR ; Channel pore ; Chloride channel ; Cystic fibrosis ; Ion selectivity
• 刊名：Cellular and Molecular Life Sciences
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：74
• 期：1
• 页码：67-83
• 全文大小：
• 刊物类别：Biomedical and Life Sciences
• 刊物主题：Cell Biology; Biomedicine, general; Life Sciences, general; Biochemistry, general;
• 出版者：Springer International Publishing
• ISSN：1420-9071
• 卷排序：74

文摘

The main function of the cystic fibrosis transmembrane conductance regulator (CFTR) is as an ion channel for the movement of small anions across epithelial cell membranes. As an ion channel, CFTR must form a continuous pathway across the cell membrane—referred to as the channel pore—for the rapid electrodiffusional movement of ions. This review summarizes our current understanding of the architecture of the channel pore, as defined by electrophysiological analysis and molecular modeling studies. This includes consideration of the characteristic functional properties of the pore, definition of the overall shape of the entire extent of the pore, and discussion of how the molecular structure of distinct regions of the pore might control different facets of pore function. Comparisons are drawn with closely related proteins that are not ion channels, and also with structurally unrelated proteins with anion channel function. A simple model of pore function is also described.