People with cystic fibrosis are at greater risk of lung infection because the thick, sticky mucus that builds up in the lungs allows germs to thrive and multiply.
All organisms – from microbes to humans – rely on protein pumps and channels that transport ions across the cell membrane. Faulty ion channels in cells underlie many currently incurable human diseases.Cystic fibrosis is caused by a genetic mutation that affects some of the proteins that make up ion channels, causing them to malfunction. The result is a thick build up of mucus in the lungs and other organs, making breathing difficult.
While treatments for cystic fibrosis exist, they do not fix the underlying cause. Treatments include inhalants, enzyme supplements and clearing the airways, which can usually only be done with help.The team tested the small molecule at low doses in a strain of yeast that cannot grow because it has faulty ion channels. When they added the molecule, the researchers found the yeast grew nearly as well as a normal strain that they used as a control.
People with cystic fibrosis have inherited two copies of the defective CF gene. About 70,000 people worldwide are living with cystic fibrosis. Most cases are diagnosed by the age of 2.