more than just a problem with chloride ions

ion transport in cystic fibrosis has important implications for treatment of this debilitating condition.

Kenneth Lee(

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 Nature, Shmuel Muallem and colleagues from the University of Texas Southwestern Medical Center, Dallas report that disruption in the transport of bicarbonate ions (HCO3-) may also be of considerable importance in CF patients (Nature 2001, 410:94-97).

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...

Interested in reading more?

Become a Member of

Receive full access to more than 35 years of archives, as well as TS Digest, digital editions of The Scientist, feature stories, and much more!
Already a member?