<i>Drosophila</i> as a model for studying cystic fibrosis pathophysiology of the gastrointestinal system
Kevin Kim, Elizabeth A. Lane, Aurelia Saftien, Haiyun Wang, Yue Xu, Frederik Wirtz‐Peitz, Norbert Perrimon
Abstract
Significance In this study, Kim et al. identify Drosophila CFTR ( dCFTR ) and show that knockdown of dCFTR in the adult intestine disrupts osmotic homeostasis and displays CF-like phenotypes that lead to intestinal stem cell hyperplasia. They show that expression of wild-type human CFTR , but not mutated forms of CFTR that affect protein production or transport, rescues the mutant phenotypes of dCFTR . Furthermore, global transcriptomic analysis of dCFTR fly intestine identified a mucin gene that is required for proper intestinal barrier protection. Altogether, their findings suggest that Drosophila can be a powerful model organism for studying CF pathophysiology.