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Review
Nature Reviews Molecular Cell Biology 7, 426–436 (1 June 2006) | doi:10.1038/nrm1949
New insights into cystic fibrosis: molecular switches that regulate CFTR
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Abstract
Cystic fibrosis transmembrane conductance regulator (CFTR), a Cl|[minus]|-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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