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Gene linked to lung disease

Gene linked to lung disease

New treatments for cystic fibrosis could soon be developed after scientists made an important discovery about a gene linked to the condition, it has been announced.

Cystic fibrosis is a hereditary disease that affects the lungs by clogging them up with mucus. Studies have shown that the IFRD1 gene controls the severity of the lethal disease.

Research, published in the journal Nature, shows that small variations of IFRD1 affected the seriousness of cystic fibrosis symptoms.

Scientists from Cincinnati Children's Hospital Medical Centre, in the US, said that deleting the gene in mice reduced lung inflammation. They found that the protein made by IFRD1 controls the function of white blood cells called neutrophils.

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

Study leader Dr Christopher Karp said: "Neutrophils appear to be particularly bad actors in cystic fibrosis. They are important to the immune system's response to bacterial infection. In cystic fibrosis, however, neutrophilic airway inflammation is dysregulated, eventually destroying the lung."

Dr Karp said that the IFRD1 gene could be a "signpost" to further studies or even a target for treatment.

Copyright © Press Association 2009

Nature

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