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New Insight into Cystic Fibrosis

Tuesday, April 26, 2016

 

 

Abnormally thick and sticky mucus that clogs airways and makes the lungs more vulnerable to persistent, difficult-to-treat bacterial infections is a major problem for people with cystic fibrosis (CF), especially in its more advanced stages.

Understanding why CF mucus is so sticky may suggest ways to more effectively treat the problem.

A recent study by researchers at the University of Iowa’s Pappajohn Biomedical Institute (PBI) suggests that CF patients’ excessively sticky mucus is present from birth, rather than a problem that develops over time. Moreover, the study suggests that this mucus is more viscous in CF patients because a thin layer of liquid that coats our airways is more acidic in those who have the condition.

The gene mutations that cause CF disrupt the function of a protein called the cystic fibrosis transmembrane conductance regulator (CFTR) channel, which secretes bicarbonate ions into the airway surface liquid. Higher levels of bicarbonate ions reduce the airway surface liquid’s acidity.

Read the complete article.