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.
AdvertisementThe 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.
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