Concepedia

R560S: A class II CFTR mutation that is not rescued by current modulators

Nikhil T Awatade, Sofia S. Ramalho, Iris A. L. Silva, Verónica Felício, Hugo M. Botelho, Eyleen de Poel, Annelotte M. Vonk, Jeffrey M. Beekman, Carlos M. Farinha, Margarida D. Amaral

Journal of Cystic Fibrosis · 2018 · 36 citations · 28 references

DOIFull text

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References

28

Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del<i>CFTR</i>

Claire Wainwright, J.S. Elborn, Bonnie W. Ramsey et al. · New England Journal of Medicine · 2015 · 1.6K citations · Full text

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

Fredrick Van Goor, Sabine Hadidaꝉ, Peter D. J. Grootenhuis et al. · Proceedings of the National Academy of Sciences · 2009 · 1.2K citations · Full text

, , +15

Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809

Fredrick Van Goor, Sabine Hadidaꝉ, Peter D. J. Grootenhuis et al. · Proceedings of the National Academy of Sciences · 2011 · 1.1K citations · Full text

A functional CFTR assay using primary cystic fibrosis intestinal organoids

Johanna F. Dekkers, Caroline L. Wiegerinck, Hugo R. de Jonge et al. · Nature Medicine · 2013 · 977 citations

, , +6

CFTR expression and chloride secretion in polarized immortal human bronchial epithelial cells.

Alison Cozens, M J Yezzi, Karl Kunzelmann et al. · American Journal of Respiratory Cell and Molecular Biology · 1994 · 930 citations

, , +19