A functional CFTR assay using primary cystic fibrosis intestinal organoids

  title={A functional CFTR assay using primary cystic fibrosis intestinal organoids},
  author={Johanna F. Dekkers and Caroline L. Wiegerinck and Hugo R. de Jonge and Inez Bronsveld and Hettie M. Janssens and Karin M de Winter-de Groot and Arianne M Brandsma and Nienke W M de Jong and Marcel J C Bijvelds and Bob J Scholte and Edward E. S. Nieuwenhuis and Stieneke van den Brink and Hans Clevers and Cornelis K. van der Ent and Sabine Middendorp and Jeffrey Matthijn Beekman},
  journal={Nature Medicine},
We recently established conditions allowing for long-term expansion of epithelial organoids from intestine, recapitulating essential features of the in vivo tissue architecture. Here we apply this technology to study primary intestinal organoids of people suffering from cystic fibrosis, a disease caused by mutations in CFTR, encoding cystic fibrosis transmembrane conductance regulator. Forskolin induces rapid swelling of organoids derived from healthy controls or wild-type mice, but this effect… CONTINUE READING
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Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508delCFTR mutation

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