The ABC protein turned chloride channel whose failure causes cystic fibrosis.
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ABSTRACT:
SUBMITTER: Gadsby DC
PROVIDER: S-EPMC2720541 | biostudies-literature | 2006 Mar
REPOSITORIES: biostudies-literature
Publications
The ABC protein turned chloride channel whose failure causes cystic fibrosis.
Gadsby David C DC Vergani Paola P Csanády László L
Nature 20060301 7083
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 chann ...[more]
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