Modeling pulmonary cystic fibrosis in a human lung airway-on-a-chip

Cystic fibrosis (CF) is a genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which encodes a membrane protein with chloride channel function [1]. In the bronchial epithelium, CFTR dysfunction causes contraction of the periciliary volume; accumulation of dense mucus, promoted by hypersecretion of the mucins MUC5AC and MUC5B and by defective mucociliary transport; chronic infection and neutrophilic inflammation, leading to respiratory insufficiency and death [2].

Source: Journal of Cystic Fibrosis - November 16, 2021 Category: Respiratory Medicine Authors: Roberto Plebani, Ratnakar Potla, Mercy Soong, Haiqing Bai, Zohreh Izadifar, Amanda Jiang, Renee N. Travis, Chaitra Belgur, Alexandre Dinis, Mark J. Cartwright, Rachelle Prantil-Baun, Pawan Jolly, Sarah E. Gilpin, Mario Romano, Donald E. Ingber Tags: Original Article Source Type: research