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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Bibliografiska uppgifter
Huvudupphovsmän: 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
Materialtyp: Artigo
Språk:Inglês
Publicerad: National Academy of Sciences 2009
Ämnen:
Biological Sciences
Länkar: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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