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Health

Approved drug may help cystic fibrosis patients

According to a news release from the University of Illinois, an approved drug normally used to treat fungal infections could also do the job of a protein channel that is missing or defective in the lungs of people with cystic fibrosis, operating as a prosthesis on the molecular scale, says new research from the University of Illinois and the University of Iowa.

Martin D. Burke is the leader of the study. Burke is a professor of chemistry at Illinois and the associate dean for research at the Carle Illinois College of Medicine. See a video of Burke explaining the findings.

Burke’s group has long investigated the channel-forming properties of a drug used to treat fungal infections, amphotericin (am-foe-TARE-is-in). In the new study, the researchers explored it as a treatment candidate for cystic fibrosis.

They found that amphotericin can form channels in the surface membrane of lung tissue donated by people with cystic fibrosis that was caused by various mutations in the CFTR gene.

The channels released bicarbonate that had built up in cells and brought the pH and thickness of the airway surface liquid back within normal range.

The researchers also treated pigs with cystic fibrosis using a version of amphotericin formulated for delivery to the lungs.

In both the experiments, in human tissue and in pigs, the researchers saw a restoration of the infection-fighting properties in the liquid lining the lung surfaces.

Cystic fibrosis is a lifelong disease that makes patients vulnerable to lung infections. There are treatments for some but not all patients, and there is no cure.

The drug restored infection-fighting properties in lung tissue donated by human patients as well as in pigs with cystic fibrosis.

It has potential to become the first treatment to address all types of cystic fibrosis, regardless of the genetic mutation that causes the protein deficiency.

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