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New insights into cystic fibrosis: molecular switches that regulate CFTR Export

Nature Reviews Molecular Cell Biology, Vol. 7, No. 6. (01 June 2006), pp. 426-436.

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bmb518 cftr golgi review trafficking

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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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