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- Title
The ABC protein turned chloride channel whose failure causes cystic fibrosis.
- Authors
Gadsby, David C; Vergani, Paola; Csanády, László
- 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.
- Publication
Nature, 2006, Vol 440, Issue 7083, p477
- ISSN
1476-4687
- Publication type
Journal Article
- DOI
10.1038/nature04712