Review
Nature Reviews Molecular Cell Biology 7, 426-436 (June 2006) | doi:10.1038/nrm1949
Subject Category: Mechanisms of disease
Article series: Mechanisms of disease
Mechanisms of disease: New insights into cystic fibrosis: molecular switches that regulate CFTR
William B. Guggino1 & Bruce A. Stanton2 About the authors
Abstract
Cystic fibrosis transmembrane conductance regulator (CFTR), a Cl--selective ion channel, is a prototypic member of the ATP-binding cassette transporter superfamily that is expressed in several organs. In these organs, CFTR assembles into large, dynamic macromolecular complexes that contain signalling molecules, kinases, transport proteins, PDZ-domain-containing proteins, myosin motors, Rab GTPases, and SNAREs. Understanding how these complexes regulate the intracellular trafficking and activity of CFTR provides a unique insight into the aetiology of cystic fibrosis and other diseases.
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Author affiliations
- Department of Physiology and Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
- Department of Physiology, Dartmouth Medical School, Hanover, New Hampshire, USA.
Correspondence to: Bruce A. Stanton2 Email: bas@Dartmouth.edu
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