606101-83-1Relevant academic research and scientific papers
CYANOQUINOLINE COMPOUNDS HAVING ACTIVITY IN CORRECTING MUTANT-CFTR PROCESSING AND INCREASING ION TRANSPORT AND USES THEREOF
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, (2013/02/28)
The present disclosure provides pharmaceutical compositions, pharmaceutical preparations and methods for increasing activity of a mutant cystic fibrosis transmembrane conductance regulator protein (mutant-CFTR). The pharmaceutical compositions, pharmaceut
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
