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

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.

SUBMITTER: Gadsby DC 

PROVIDER: S-EPMC2720541 | biostudies-literature | 2006 Mar

REPOSITORIES: biostudies-literature