Cystic Fibrosis - New Finding

In a recent study it was found that cystic fibrosis is caused by a genetic defect in a regulator due to the lack of a key protein.

The study led by Daniela Rotin, PhD, senior scientist at SickKids and professor of biochemistry at the University of Toronto, showed that the defect in a chloride channel, called cystic fibrosis transmembrane conductase regulator (CFTR), is caused by the lack of a protein called ubiquitin ligase Nedd4L.

The scientists experimented on mice specially bred to lack Nedd4L in the lung and discovered that they developed cystic fibrosis-like lung diseases, including inflammation and obstructed airways, and died within 3 weeks of birth.

Another way to treat the disease might be to suppress epithelial sodium channel (ENaC) - the protein that leads lungs to absorb excessive of salt.

Source: ANI