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New proteins to clear the airways in CF

[Posted: Mon 16/07/2012 by Gillian Tsoi www.irishhealth.com]

Scientists in the US have uncovered a new strategy that may one day help people with cystic fibrosis and chronic obstructive pulmonary disorder (COPD) better clear the thick and sticky mucus that clogs their lungs and leads to life-threatening infections.

Researchers at the University of North Carolina have shown that the "SPLUNC1" protein may be able to help thin this thick mucus by affecting the epithelial sodium channel (ENaC).

ENaC plays an important role in clearing liquid and regulating of mucous fluidity in the lungs and airways.

Not only does this research have implications for cystic fibrosis and COPD, but it also enhances the understanding of hypertension due to the role it also plays in controlling blood pressure.

"We hope that this study will pave the way for a new class of peptide-based channel inhibitors that can help reverse the mucus dehydration seen in Cystic Fibrosis and COPD," said Dr Robert Tarran, a researcher involved in the work from the Cystic Fibrosis/Pulmonary Research and Treatment Center at the University of North Carolina in Chapel Hill. "This would help restore mucus clearance and kick-start the lung's ability to clear unwanted pathogens."

Cystic fibrosis is an inherited disease that affects the lungs, digestive system, sweat glands and male fertility. The condition affects the body's ability to move salt and water in and out of cells. This defect causes the lungs and pancreas to secrete abnormally thick mucus that blocks passageways and prevents proper function.

COPD is a lung disease characterized by chronic obstruction of lung airflow that interferes with normal breathing.

"Breathing is something most healthy people take for granted," said Dr Gerald Weissmann, editor-in-chief of The FASEB Journal. "However, people with cystic fibrosis and COPD battle for every breath because sticky mucus plugs their airways. This research should give scientists a new way of clearing the air for people with cystic fibrosis and COPD."

The research was published online in the FASEB Journal.

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