Article
Ibuprofen rescues mutant cystic fibrosis transmembrane conductance regulator trafficking.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Jan 2015
Carlile Graeme W, Robert Renaud, Goepp Julie, Matthes Elizabeth, Liao Jie, Kus Bart, Macknight Sean D, Rotin Daniela, Hanrahan John W, Thomas David Y
Abstract excerpt
BACKGROUND: Small molecules as shown by VX809 can rescue the mislocalization of F508del-CFTR. The aim of this study was to identify correctors with a clinical history and their targets of action. METHODS: CFTR correctors were screened using two F508del-CFTR expressing cell based HTS assays. Electrophysiological studies using CFBE41o(-) and HBE cells and in-vivo mouse assays confirmed CFTR rescue. The target of...
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