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Inner workings of cystic fibrosis.

Inner workings of cystic fibrosis

Cystic fibrosis, the most common fatal genetic disease in Caucasians, is beginning to reveal its secrets. Last year, geneticists found chromosomal markers for the disease (SN:10/19/85, p.244). Now it is the biochemists' turn: Researchers have found a defect in cell function of people with cystic fibrosis.

Since Paul Quinton of the University of California at Riverside discovered in 1983 that sweat gland cells of cystic fibrosis victims are not very permeable to chloride ions, researchers have focused on the channels that carry chloride across cell membranes. Now they have shown the problem to be at the level of what controls the channel, not at that of the channels themselves, according to a report in the Aug. 1 SCIENCE by researchers at the University of Alabama at Birmingham and in the July 31 NATURE by researchers at the University of Iowa in Iowa City and Case Western Reserve University in Cleveland.

Normal chloride movement pulls water from the tissues to the lung lining; without this water, mucus in the lungs is too thick and sticky, and interferes with normal lung function. One in 2,000 U.S. Caucasians is born with the disease, and half die by age 21. Current therapy consists of chest pounding to loosen the lung secretions, and anti-biotics for the frequent lung infections.

The two groups independently found that the channels in cystic fibrosis patients' cells failed to respond to a chemical that usually stimulates chloride movement. The channels sit in the cell membranes, and when the cells were disrupted and just the membranes were tested, the channels responded properly, indicating that the problem lies in the cell's control over the channel.

"Nothing's different tomorrow from what it was yesterday for cystic fibrosis patients,' says Iowa's Michael J. Welsh. "But if we can find out the basic defect, we might be able to develop a rational therapy.'
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Author:Silberner, Joanne
Publication:Science News
Date:Aug 9, 1986
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