The ABC protein turned chloride channel whose failure causes cystic fibrosis

David C. Gadsby, Paola Vergani, L. Csanády

Research output: Contribution to journalArticle

462 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)477-483
Number of pages7
JournalNature
Volume440
Issue number7083
DOIs
Publication statusPublished - Mar 23 2006

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Chloride Channels
Cystic Fibrosis
Adenosine Triphosphate
ATP-Binding Cassette Transporters
Active Biological Transport
Anions
Adenosine Triphosphatases
Proteins
Membranes
Genes

ASJC Scopus subject areas

  • General

Cite this

The ABC protein turned chloride channel whose failure causes cystic fibrosis. / Gadsby, David C.; Vergani, Paola; Csanády, L.

In: Nature, Vol. 440, No. 7083, 23.03.2006, p. 477-483.

Research output: Contribution to journalArticle

Gadsby, David C. ; Vergani, Paola ; Csanády, L. / The ABC protein turned chloride channel whose failure causes cystic fibrosis. In: Nature. 2006 ; Vol. 440, No. 7083. pp. 477-483.
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