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Nature. 2006 Mar 23;440(7083):477-83.

The ABC protein turned chloride channel whose failure causes cystic fibrosis.

Author information

  • 1Laboratory of Cardiac/Membrane Physiology, The Rockefeller University, New York, NY 10021, USA. gadsby@rockefeller.edu

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.

PMID:
16554808
[PubMed - indexed for MEDLINE]
PMCID:
PMC2720541
Free PMC Article

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