Cystic fibrosis transmembrane conductance regulator (CFTR): Making an ion channel out of an active transporter structure

Channels
Paul Linsdell

Abstract

Cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR). CFTR is a member of the ATP-binding cassette (ABC) family of membrane transport proteins, most members of which function as ATP-dependent pumps. CFTR is unique among human ABC proteins in functioning not as a pump, but as an ion channel. Recent structural data has indicated that CFTR shares broadly similar overall architecture and ATP-dependent conformational changes as other ABC proteins. Functional investigations suggest that CFTR has a unique open portal connecting the cytoplasm to the transmembrane channel pore, that allows for a continuous pathway for Cl- ions to cross the membrane in one conformation. This lateral portal may be what allows CFTR to function as an ion channel rather than as a pump, suggesting a plausible mechanism by which channel function may have evolved in CFTR.

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Citations

Feb 26, 2019·Microbial Biotechnology·Grégory BoëlAntoine Danchin
Feb 13, 2020·International Journal of Molecular Sciences·Francesca ChiariniCamilla Evangelisti
Jan 2, 2020·Cell Biochemistry and Biophysics·Paul LinsdellYassine El Hiani
Nov 17, 2020·European Journal of Medicinal Chemistry·Margarida D Amaral
Jan 15, 2021·Biochimica Et Biophysica Acta. Biomembranes·Paul Linsdell
Nov 9, 2020·Biochemical Pharmacology·Amir Pelleg
Jun 3, 2021·Molecules : a Journal of Synthetic Chemistry and Natural Product Chemistry·Aleksandra SekPiotr Bednarczyk

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Methods Mentioned

BETA
electron cryomicroscopy

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