Peter van Mourik

136 Chapter 6 8. Hirtz S, Gonska T, Seydewitz HH, Thomas J, Greiner P, Kuehr J, Brandis M, Eichler I, Rocha H, Lopes A, Barreto C, Ramalho A, Amaral MD, Kunzelmann K, Mall M. CFTR Cl− channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis. Gastroenterology 2004;127:1085– 1095. 9. Mall M, Kreda SM, Mengos A, Jensen TJ, Hirtz S, Seydewitz HH, Yankaskas J, Kunzelmann K, Riordan JR, Boucher RC. The DeltaF508 mutation results in loss of CFTR function and mature protein in native human colon. Gastroenterology 2004;126:32–41. 10. Wainwright CE, Elborn JS, Ramsey BW, Marigowda G, Huang X, Cipolli M, Colombo C, Davies JC, de BK, Flume PA, Konstan MW, McColley SA, McCoy K, McKone EF, Munck A, Ratjen F, Rowe SM, Waltz D, Boyle MP. Lumacaftor- Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR. N Engl J Med 2015. 11. Middleton PG, Mall MA, Dřevínek P, Lands LC, McKone EF, Polineni D, Ramsey BW, Taylor-Cousar JL, Tullis E, Vermeulen F, Marigowda G, McKee CM, Moskowitz SM, Nair N, Savage J, Simard C, Tian S, Waltz D, Xuan F, Rowe SM, Jain R. Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele. N Engl J Med 2019;381:1809–1819.