Article
Forskolin-induced swelling of intestinal organoids correlates with disease severity in adults with cystic fibrosis and homozygous F508del mutations.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Jul 2020
de Winter-de Groot Karin M, Berkers Gitte, Marck-van der Wilt Rozemarijn E P, van der Meer Renske, Vonk Annelotte, Dekkers Johanna F, Geerdink Margot, Michel Sabine, Kruisselbrink Evelien, Vries Rob, Clevers Hans, Vleggaar Frank P, Elias Sjoerd G, Heijerman Harry G M, van der Ent Cornelis K, Beekman Jeffrey M
Abstract excerpt
BACKGROUND: CFTR function measurements in intestinal organoids may help to better characterise individual disease expression in F508del homozygous people. Our objective was to study correlations between CFTR function as measured with forskolin-induced swelling in rectal organoids with clinical parameters in adult patients with homozygous F508del mutations. METHODS: Multicentre observational study. Thirty-four...
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