New insights into cystic fibrosis: molecular switches that regulate CFTR.
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.
Guggino
William B
WB
Department of Physiology and Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Stanton
Bruce A
BA
D003550:Cystic Fibrosis / Q000378:metabolism*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000378:metabolism*; D006801:Humans; D007473:Ion Channels / Q000378:metabolism*; D021381:Protein Transport
D007473:Ion Channels; D019005:Cystic Fibrosis Transmembrane Conductance Regulator