Mouse intestine as a useful model for CFTR electrophysiology function analysis.

Abstract

Cystic fibrosis (CF) is a genetic disorder primarily known for its severe impact on lung function, but it also significantly affects the digestive system, leading to complications such as intestinal blockages, malabsorption, inflammation, and microbial dysbiosis. The study of CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) effects on intestinal physiology is critical for developing new effective treatments. This work highlights the use of the mouse intestine as a valuable model for analyzing cellular electrophysiology and CFTR function. The method reviews the molecular mechanisms underlying intestinal absorption in CF and the role of CFTR in maintaining gut homeostasis. The transgenic mouse models mimicking CF features allow researchers to explore drug responses, and potential therapeutic interventions by studying the molecular mechanisms involved, such as inflammation, autophagy or immunity. The Ussing chamber assay is a versatile tool for evaluating ion transport, barrier function, and the effects of drugs on intestinal epithelial integrity. This assay provides insights into CFTR functionality and the influence of various compounds on intestinal absorption and permeability. This protocol underscores the importance of understanding CF's gastrointestinal implications emphasizing the potential for mouse models to advance therapeutic strategies not only for CF but also for other gastrointestinal disorders.