Peter van Mourik

201 General discussion 79. Hirtz, S. et al. CFTR Cl− channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis. Gastroenterology 127 , 1085–1095 (2004). 80. Sousa, M. et al. Measurements of CFTR-mediated Cl- secretion in human rectal biopsies constitute a robust biomarker for Cystic Fibrosis diagnosis and prognosis. PLoS One 7 , e47708 (2012). 81. Graeber, S. Y. et al. Effects of Lumacaftor-Ivacaftor Therapy on Cystic Fibrosis Transmembrane Conductance Regulator Function in Phe508del Homozygous Patients with Cystic Fibrosis. Am. J. Respir. Crit. Care Med. 197 , 1433–1442 (2018). 82. Neuberger, T., Burton, B., Clark, H. & Van Goor, F. Use of Primary Cultures of Human Bronchial Epithelial Cells Isolated from Cystic Fibrosis Patients for the Pre-clinical Testing of CFTR Modulators. in 39–54 (2011). doi:10.1007/978-1- 61779-117-8_4. 83. Brewington, J. J. et al. Brushed nasal epithelial cells are a surrogate for bronchial epithelial CFTR studies. JCI Insight 3 , (2018). 84. Pranke, I. M. et al. Correction of CFTR function in nasal epithelial cells from cystic fibrosis patients predicts improvement of respiratory function by CFTR modulators. Sci. Rep. 7 , 7375 (2017). 9