Genetic breakthrough on cystic fibrosis

Published on Wednesday 25 February 2009 22:15

A GENE discovery could pave the way to new treatments for cystic fibrosis, scientists reported last night.

The gene, called IFRD1, controls the severity of the lethal, inherited disease, which clogs up and destroys the lungs.

A genetic study showed small variations of IFRD1 affected the seriousness of cystic fibrosis symptoms in almost 3,000 human patients.

In mice with the disease, deleting the gene reduced lung inflammation. Scientists discovered the protein made by IFRD1 regulates the function of white blood cells called neutrophils.

The cells, part of the immune system, are known to cause inflammatory damage to the airways of people with cystic fibrosis.

The research showed that removing IFRD1 blunted the inflammatory response of neutrophils, but did not impair the overall functioning of the immune system.