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New Insights Into Cystic Fibrosis Ion Channel

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Science  15 Oct 1999:
Vol. 286, Issue 5439, pp. 388-389
DOI: 10.1126/science.286.5439.388

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Summary

The gene defective in cystic fibrosis encodes a protein that channels chloride ions through the cell membrane, thereby regulating the water and salt balance in cells that line organs such as the lungs and intestines. On page 544, researchers report that they have identified a particular section on the tail of this protein, called the cystic fibrosis transmembrane conductance regulator (CFTR), that is needed to help keep the channel open. That CFTR region thus becomes a good target for drugs aimed at regulating the channel opening. Those that enhance the opening might help some cystic fibrosis patients. In addition, since the watery diarrhea caused by the cholera bacterium and other pathogens is due to toxins that kick the CFTR into overdrive, drugs that inhibit chloride transport through the channel might help the much larger number of persons suffering from such infections.

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