604754-35-0Relevant articles and documents
Structure-activity relationships of cyanoquinolines with corrector-potentiator activity in Δf508 cystic fibrosis transmembrane conductance regulator protein
Knapp, John M.,Wood, Alex B.,Phuan, Puay-Wah,Lodewyk, Michael W.,Tantillo, Dean J.,Verkman,Kurth, Mark J.
, p. 1242 - 1251 (2012/04/04)
Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel. The most common CF-causing mutation, ΔF508-CFTR, produces CFTR loss-of-function by impairing its cellular targeting to the plas
Cyanoquinolines with independent corrector and potentiator activities restore ΔPhe508-cystic fibrosis transmembrane conductance regulator chloride channel function in cystic fibrosis
Phuan, Puay-Wah,Yang, Baoxue,Knapp, John M.,Wood, Alex B.,Lukacs, Gergely L.,Kurth, Mark J.,Verkman, Alan S.
, p. 683 - 693 (2012/06/04)
The ΔPhe508 mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) protein impairs its folding, stability, and chloride channel gating. Although small molecules that separately correct defective ΔPhe508-CFTR folding/cellular processing