Thursday 26 February 2009

Interesting news story about CF

You may have seen in the news today some interesting research about CF. Scientists have discovered that small differences in a gene called IFRD1 in people with CF can relate to the severity of lung disease in the patient.

Here's some more info

We asked the scientists at the UK CF Gene Therapy Consortium what they thought about this and here's what they said.

"It has been suspected for a long time that the severity of cystic fibrosis (CF) lung disease is influenced by genes other than CFTR (the "CF gene") in CF patients. By examining the genetic make-up of a large group of patients, Gu and colleagues have identified IFRD1 as having a
significant effect on lung disease: some forms of the gene are correlated with lower severity and other forms with higher severity. Interestingly, IFRD1 affects the function of neutrophils, cells of the immune system which are responsible for much of the damage seen in the lungs of patients.

The result is important because it confirms the importance of other genes in modulating CF lung disease and implies that therapies aimed at modulating neutrophil function would have a beneficial effect in CF. Such therapies could eventually become a useful addition to the existing
range of symptomatic therapies available. However, therapies aimed at correcting the basic defect in CF still offer the best prospect of providing long-term clinical benefit."

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