Cystic fibrosis (CF) is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR), which forms a chloride ion (Cl-) transport channel in epithelial cells. But the existence of some CF patients with normal Cl- ion channel activity has puzzled researchers for some time.
In 1 March
They measured HCO3- and Cl- transport in cells transfected with DNA encoding wild-type or mutant CFTR. In 11 common mutations of CFTR, the transport of HCO3- was more greatly impaired than that of Cl-.
It is lung disease that accounts for most of the mortality in...