

Cystic fibrosis scientists discover abnormal response to lung infections

The inability to clear bacteria from the lungs is the result of a lack of healthy mucus secreted in the airways of those with cystic fibrosis, according to new research.

“For a very long time, there has been discussion about whether cystic fibrosis was a bacteria-infection problem, an inflammation problem, or an immune system problem,” said Juan Ianowski, the lead author of the paper published today in the prestigious Nature Communications journal.


“Most patients are born with healthy lungs and as time passes, they start developing infections and inflammation. The infections are cleared and a new one occurs, and then there is an infection and it’s cleared and a new one occurs. Eventually, the infections are permanent and the bacteria never leave the lungs,” said Ianowski.

Cystic Fibrosis Canada estimates that one in every 3,600 children born in Canada has cystic fibrosis. It is the most common fatal genetic disease affecting Canadian children and young adults, according to the organization’s website.

People with healthy lungs inhale between 5,000 and 14,000 germs every day. These germs are caught in their airways by a sticky mucus with antimicrobial properties that kills the germs. Tiny hairs called cilia then push the germs out of the respiratory tract and the body clears the mucus and the germs.


Ianowski and his team spent seven years developing the synchrotron-based method needed to analyze the production of mucus in a living animal. The normal layer of mucus is as thin as 80 microns. That’s thinner than a strand of hair.


“We discovered and showed for the first time ever that the normal airway response to inhalation of pathogens is producing liquid. This is a process that depends on CFTR (cystic fibrosis transmembrane conductance regulator) expression. So if you don’t have normal CFTR, it doesn’t work. In CF pigs, this response is absent,” explained Ianowski.

Read more at the Canadian Light Source

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