Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

Cystic fibrosis (CF) is a fatal genetic disease caused by mutations in the gene encoding the CF transmembrane conductance regulator (CFTR), a protein kinase A (PKA)-activated epithelial anion channel involved in salt and fluid transport in multiple organs, including the lung. Most CF mutations eithe...

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Detalhes bibliográficos
Main Authors: Van Goor, Fredrick, Hadida, Sabine, Grootenhuis, Peter D. J., Burton, Bill, Cao, Dong, Neuberger, Tim, Turnbull, Amanda, Singh, Ashvani, Joubran, John, Hazlewood, Anna, Zhou, Jinglan, McCartney, Jason, Arumugam, Vijayalaksmi, Decker, Caroline, Yang, Jennifer, Young, Chris, Olson, Eric R., Wine, Jeffery J., Frizzell, Raymond A., Ashlock, Melissa, Negulescu, Paul
Formato: Artigo
Idioma:Inglês
Publicado em: National Academy of Sciences 2009
Assuntos:
Biological Sciences
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2773991/
https://ncbi.nlm.nih.gov/pubmed/19846789
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.0904709106
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