Forskolin-induced swelling of intestinal organoids correlates with disease severity in adults with cystic fibrosis and homozygous F508del mutations

FIS of intestinal organoids varied between subjects with homozygous F508del and correlated with pulmonary and nutritional parameters. These findings suggest that differences at low CFTR residual function may contribute to clinical heterogeneity in F508del homozygous patients and small changes in CFTR residual function might impact long-term disease expression.

Source: Journal of Cystic Fibrosis - November 13, 2019 Category: Respiratory Medicine Authors: Karin M. de Winter – de Groot, Gitte Berkers, Rozemarijn E.P. Marck – van der Wilt, Renske van der Meer, Annelotte Vonk, Johanna F. Dekkers, Margot Geerdink, Sabine Michel, Evelien Kruisselbrink, Rob Vries, Hans Clevers, Frank P. Vleggaar, Sjoerd G. E Tags: Original Article Source Type: research