Cystic fibrosis (CF) is a recessive genetic disease caused by mutations in the CFTR gene (Cystic fibrosis transmembrane conductance regulator), a protein with chloride channel function. Key consequences of a CFTR dysfunction include imbalance in ion transport, mucus accumulation, and chronic inflammation as well as infection in the airways of CF patients.
In this webinar, Roberto Plebani, PhD, described how he used the Chip-S1® Stretchable Chip to develop the first CF Airway Lung-Chip using primary cells derived from patients’ airways and lung microvascular endothelial cells. The chip not only allowed the reconstruction of a CF bronchial unit, with increased mucus production and inflammation, but also enabled the perfusion of immune cells and infection with pathogens to better model inflammation and infection in CF.
Key highlights from the webinar include:
- Development of the first CF Airway Lung-Chip and potential enhancements for its use as a preclinical CF model.
- How the chip replicates a CF bronchial unit with increased mucus, inflammation, immune cell perfusion, and pathogen infection to better model CF.
- Future studies that will use the chip for drug testing and aim to improve it by adding CF endothelium and stroma.