Article
Potentiation of ΔF508- and G551D-CFTR-Mediated Cl- Current by Novel Hydroxypyrazolines.
PloS one - 1 Jan 2016
Park Jinhong, Khloya Poonam, Seo Yohan, Kumar Satish, Lee Ho K, Jeon Dong-Kyu, Jo Sungwoo, Sharma Pawan K, Namkung Wan
Abstract excerpt
The most common mutation of CFTR, affecting approximately 90% of CF patients, is a deletion of phenylalanine at position 508 (F508del, ΔF508). Misfolding of ΔF508-CFTR impairs both its trafficking to the plasma membrane and its chloride channel activity. To identify small molecules that can restore channel activity of ΔF508-CFTR, we synthesized and evaluated eighteen novel hydroxypyrazoline analogues as CFTR...
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