TY - JOUR
T1 - New insights into cystic fibrosis
T2 - Molecular switches that regulate CFTR
AU - Guggino, William B.
AU - Stanton, Bruce A.
PY - 2006/6
Y1 - 2006/6
N2 - 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.
AB - 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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U2 - 10.1038/nrm1949
DO - 10.1038/nrm1949
M3 - Review article
C2 - 16723978
AN - SCOPUS:33745772850
SN - 1471-0072
VL - 7
SP - 426
EP - 436
JO - Nature Reviews Molecular Cell Biology
JF - Nature Reviews Molecular Cell Biology
IS - 6
ER -