The ABC Protein Turned Chloride Channel Whose Failure Causes Cystic Fibrosis

Overview

Journal Nature

Specialty Science

Date 2006 Mar 24

PMID 16554808

Citations 307

Authors

David C Gadsby

Paola Vergani

Laszlo Csanady

Affiliations

Soon will be listed here.

Abstract

CFTR chloride channels are encoded by the gene mutated in patients with cystic fibrosis. These channels belong to the superfamily of ABC transporter ATPases. ATP-driven conformational changes, which in other ABC proteins fuel uphill substrate transport across cellular membranes, in CFTR open and close a gate to allow transmembrane flow of anions down their electrochemical gradient. New structural and biochemical information from prokaryotic ABC proteins and functional information from CFTR channels has led to a unifying mechanism explaining those ATP-driven conformational changes.

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