Restoring airway integrity in cystic fibrosis patients

Hydration restores the seal at the top of the airway. In turquoise, the interaction between epithelial cells (blue). Credit: UNIGE – Laboratory by Professor Marc Chanson

Cystic fibrosis is a rare genetic disease that can cause severe symptoms. Patients in particular are suffering from chronic obstructive pulmonary disease which causes respiratory failure. It is produced by mutations in the CFTR cell, which regulates fluid flow in the cell. Therefore, when the quality of the flow changes, it can not carry unwanted bacteria and expel them. Using a model that stimulates the respiratory epithelium – a protective layer consisting of a double layer – groups from the University of Geneva (UNIGE) have discovered that a simple water film it is sufficient to seal the airways and reduce the risk of infection. . These results are published in a special issue of this journal Organic matter, paving the way to new therapies related to the hydration of mucus. A good alternative to current therapies that are usually less effective.

Despite recent medical advances, people have cystic fibrosis-one in every 2,500 births in Europe – it is longevity who is less than 46 years old and has changed his lifestyle. The disease results in one or more mutations in the CFTR gene, which affects the proper function of the immune system. The epithelial cells that line the airways are usually closed together and therefore protect the airways from bacterial overgrowth. They are also lined with a slippery liquid that catches unwanted bacteria. When the CFTR protein is modified, the interaction between the cells is relaxed and the hydrated tissue is trying to weaken, both of which promote the development of respiratory diseases.

“While it is already known that hydration of internal organs and the presence of adequate accommodation have maintained the integrity of the airways, the mechanisms involved in the integration of these two systems have been remarkable, which has prevented the development of new therapies,” he said. said Marc Chanson, professor in the Department of Molecular Biology and Metabolism and the Geneva Center for Disease Control at UNIGE Faculty of Medicine, who led the study.

Water to restore compression

Scientists began to produce in vitro specimens using human lung tissue. This product, which was awarded the UNIGE 3R award in 2021 for reducing animal testing, regenerates the airway epithelium of patients with cystic fibrosis in an appropriate manner and is close to clinical reality. In collaboration with Christian van Delden and Thilo Köhler from the Department of Medicine and Microbiology and Pharmacology at the UNIGE Faculty of Medicine, Marc Chanson and his team outlined the response. Epithelial cells which is ineffective for CFTR to bacterial infection, which is either supplemented with water, healthy fats or physiological saline solution.

Juliette Simonin, a graduate student in the Marc Chanson laboratory and lead author of the study said: “We have observed a similar response in two cases: the presence of water, whatever it is, returns airways and protect them from infection. ” “Surface hydration is sufficient to strengthen the interaction between cells and protect the epithelium’s integrity from colonization, even when CFTR is inactive.”

One solution for all mutations?

Triple treatment with drugs targeting CFTR protein has recently become available in the market. However, it only targets certain CFTR mutations and is restricted to a specific number of people with cystic fibrosis. There are still no effective and safe medicines.

“Our results provide evidence that recycling of space water is beneficial. The challenge now is to find an easy way to do this in all people suffering from the disease, whatever the replacement involves, says Marc Chanson.

Cystic fibrosis: Why so many respiratory problems?

Learn more:
Juliette L. Simonin et al, Surface Hydration Protects Cystic Fibrosis Airways from Infection by Restoration of Junctional Networks, Organic matter (2022). DOI: 10.3390 / cells11091587

hintRestoration of airway integrity in patients with cystic fibrosis (2022, May 24) was restored May 24, 2022 from

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