@article {1504570,
	title = { as a model for studying cystic fibrosis pathophysiology of the gastrointestinal system},
	journal = {Proc Natl Acad Sci U S A},
	volume = {117},
	number = {19},
	year = {2020},
	month = {2020 05 12},
	pages = {10357-10367},
	abstract = {Cystic fibrosis (CF) is a recessive disease caused by mutations in the  () gene. The most common symptoms include progressive lung disease and chronic digestive conditions. CF is the first human genetic disease to benefit from having five different species of animal models. Despite the phenotypic differences among the animal models and human CF, these models have provided invaluable insight into understanding disease mechanisms at the organ-system level. Here, we identify a member of the ABCC4 family, CG5789, that has the structural and functional properties expected for encoding the  equivalent of human CFTR, and thus refer to it as  (). We show that knockdown of  in the adult intestine disrupts osmotic homeostasis and displays CF-like phenotypes that lead to intestinal stem cell hyperplasia. We also show that expression of wild-type human , but not mutant variants of CFTR that prevent plasma membrane expression, rescues the mutant phenotypes of  Furthermore, we performed RNA sequencing (RNA-Seq)-based transcriptomic analysis using  fly intestine and identified a mucin gene, , which is required for proper intestinal barrier protection. Altogether, our findings suggest that  can be a powerful model organism for studying CF pathophysiology.},
	keywords = {Animals, Cystic Fibrosis, Cystic Fibrosis Transmembrane Conductance Regulator, Disease Models, Animal, Drosophila melanogaster, Drosophila Proteins, High-Throughput Nucleotide Sequencing, Homeostasis, Humans, Intestines, Mucins, Mutation, Phenotype, Stem Cells},
	issn = {1091-6490},
	doi = {10.1073/pnas.1913127117},
	author = {Kim, Kevin and Lane, Elizabeth A and Saftien, Aurelia and Wang, Haiyun and Xu, Yue and Wirtz-Peitz, Frederik and Perrimon, Norbert}
}
