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The ABC protein turned chloride channel whose failure causes cystic fibrosis

Author

Listed:
  • David C. Gadsby

    (Laboratory of Cardiac/Membrane Physiology, The Rockefeller University)

  • Paola Vergani

    (University College London)

  • László Csanády

    (Semmelweis University, 1444 Budapest, Pf. 262)

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.

Suggested Citation

  • David C. Gadsby & Paola Vergani & László Csanády, 2006. "The ABC protein turned chloride channel whose failure causes cystic fibrosis," Nature, Nature, vol. 440(7083), pages 477-483, March.
    • Handle: RePEc:nat:nature:v:440:y:2006:i:7083:d:10.1038_nature04712
    DOI: 10.1038/nature04712
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    Cited by:

    1. Xiaolong Gao & Han-I Yeh & Zhengrong Yang & Chen Fan & Fan Jiang & Rebecca J. Howard & Erik Lindahl & John C. Kappes & Tzyh-Chang Hwang, 2024. "Allosteric inhibition of CFTR gating by CFTRinh-172 binding in the pore," Nature Communications, Nature, vol. 15(1), pages 1-13, December.

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