In vitro 3D culture lung model from expanded primary cystic fibrosis human airway cells

Cystic fibrosis (CF) is a monogenic disease affecting the human respiratory, digestive and reproductive systems. Caused by over 2,000 genetic mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, airway symptoms include excessive production of thick mucus in the lungs, which can lead to pathogenic infections and ultimately lung damage and respiratory failure. The most common mutation is deletion of phenylalanine at position 508 (F508del; present in ~85% of individuals with CF) [1].

Source: Journal of Cystic Fibrosis - June 17, 2020 Category: Respiratory Medicine Authors: Rachael E. Rayner, Jack Wellmerling, Wissam Osman, Sean Honesty, Maria Alfaro, Mark E. Peeples, Estelle Cormet-Boyaka Source Type: research