The ABC protein turned chloride channel whose failure causes cystic fibrosis

David C. Gadsby, Paola Vergani, L. Csanády

Research output: Article

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 - márc. 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: Article

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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