Maximization of the rate of chloride conduction in the CFTR channel pore by ion-ion interactions

Xiandi Gong; Paul Linsdell

doi:10.1016/j.abb.2004.03.033

Maximization of the rate of chloride conduction in the CFTR channel pore by ion-ion interactions

Xiandi Gong, Paul Linsdell

Medicine

Research output: Contribution to journal › Article › peer-review

23 Citations (Scopus)

Abstract

Multi-ion pore behaviour has been identified in many Cl^- channel types but its biophysical significance is uncertain. Here, we show that mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) Cl^- channel that disrupt anion-anion interactions within the pore are associated with drastically reduced single channel conductance. These results are consistent with models suggesting that rapid Cl^- permeation in CFTR results from repulsive ion-ion interactions between Cl^- ions bound concurrently inside the pore. Naturally occurring mutations that disrupt these interactions can result in cystic fibrosis.

Original language	English
Pages (from-to)	78-82
Number of pages	5
Journal	Archives of Biochemistry and Biophysics
Volume	426
Issue number	1
DOIs	https://doi.org/10.1016/j.abb.2004.03.033
Publication status	Published - Jun 1 2004

Bibliographical note

Funding Information:
We thank Susan Burbridge for technical assistance. This work was supported by the Canadian Institutes of Health Research and the Canadian Cystic Fibrosis Foundation (CCFF). X. Gong is a CCFF postdoctoral fellow. P. Linsdell is a CCFF scholar.

ASJC Scopus Subject Areas

Biophysics
Biochemistry
Molecular Biology

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abstract = "Multi-ion pore behaviour has been identified in many Cl- channel types but its biophysical significance is uncertain. Here, we show that mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel that disrupt anion-anion interactions within the pore are associated with drastically reduced single channel conductance. These results are consistent with models suggesting that rapid Cl- permeation in CFTR results from repulsive ion-ion interactions between Cl- ions bound concurrently inside the pore. Naturally occurring mutations that disrupt these interactions can result in cystic fibrosis.",

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AB - Multi-ion pore behaviour has been identified in many Cl- channel types but its biophysical significance is uncertain. Here, we show that mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel that disrupt anion-anion interactions within the pore are associated with drastically reduced single channel conductance. These results are consistent with models suggesting that rapid Cl- permeation in CFTR results from repulsive ion-ion interactions between Cl- ions bound concurrently inside the pore. Naturally occurring mutations that disrupt these interactions can result in cystic fibrosis.

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Maximization of the rate of chloride conduction in the CFTR channel pore by ion-ion interactions

Abstract

Bibliographical note

ASJC Scopus Subject Areas

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