CF protein also a culprit in emphysema

Here's an example of how research into the causes of one disease can have unexpected applications in another. A team from Johns Hopkins University has found that a protein well known to be involved in cystic fibrosis can also be involved in emphysema and possibly other lung diseases. When the protein called cystic fibrosis transmembrane conductance regulator (CFTR) is working as it should, transporting chloride in and out of cells, everything is fine. But when a genetic mutation blocks that function, the result is a thick mucus buildup in the lungs and the symptoms of cystic fibrosis.

Writing in the December issue of The Journal of Immunology, the Johns Hopkins team found that CFTR plays a wider role than once believed, and according to the new research the level of CFTR corresponds also to the severity of lung damage due to emphysema.

"Our findings suggest that CFTR is a multi-tasker protein that is not only involved in chloride transport but also in regulating cell death and inflammation by keeping in check the rampant and dangerous accumulation of ceramide," principal investigator Neeraj Vij, a pulmonary researcher at Hopkins Children's and assistant professor at the Johns Hopkins University School of Medicine, is quoted as saying in a report in Drug Discovery & Development.

Vij says that the level of CFTR might be a good predictor of susceptibility lung damage due to smoking and infections.

- read the report in Drug Discovery & Development

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