Cystic fibrosis is a catastrophic disease caused by mutations in the CFTR gene. However, not all patients with cystic fibrosis have the same symptoms or the same reactions to drugs. In a recent study, researchers at the University of Cambridge and Yale University have developed a new direct method for testing multiple drugs in the cells of patients with cystic fibrosis.
This test combines a high-speed video microscope with a new video analysis algorithm to measure the coordinated movement of ciliary structures, which are arranged in cells of the respiratory tract to remove mucus from the lungs and upper respiratory tract. In patients with cystic fibrosis, the respiratory tract is concentrated in the mucus layer, causing chronic infection, obstructing breathing and interfering with normal cilia movement.
The research team used their tests to study the movement of ciliary hairs on the cell surface of polycystic fibrosis patients with different genetic mutations, and compared these samples with normal cells. We then measured the response of these cells to six different drug treatments, including drugs that are not currently approved for the treatment of cystic fibrosis. Researchers found that patients responded differently to the drug. This suggests that even if everyone has the same mutation, a drug that works for one person may not work for another.
Looking to the future, the researchers hope that their test can be used to promote more personalized treatment of this life-threatening disease. Maurizio Chioccioli is a PhD researcher in pulmonary science, intensive care and sleep medicine at Yale University. The first author of the paper was published in Nature Communications.