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Immune Proteins Give Clues to Cystic Fibrosis

Three show up in CF patients in unique way, researchers say

FRIDAY, Nov. 3, 2006 (HealthDay News) -- British scientists have discovered a possible link between certain proteins and cystic fibrosis, a genetic disease that attacks the lungs and other organs and significantly shortens life expectancy.

The University of Sheffield researchers found a highly unusual distribution of the proteins -- SPLUNC1, SPLUNC2 and LPLUNC1 -- in the lungs and airways of cystic fibrosis patients. The proteins are believed to play a role in the immune system.

The finding could improve scientists' understanding about the immune system's role in cystic fibrosis and about how the disease progresses.

In this study, the researchers compared lung tissue from 21 cystic fibrosis patients to healthy lung tissue from 10 other people.

"Our results show unique expression domains for (the proteins) within the airways and suggest that alterations in expression of these putative innate immune molecules may be associated with lung disease," the study authors wrote.

"We've shown these proteins to be expressed in places like the upper airways, nose and mouth, where many bacteria and infectious agents are found," Lynne Bingle, of the School of Clinical Dentistry at the University of Sheffield, said in a prepared statement.

It's believed that these proteins are part of the first line of the body's defenses against infectious agents, she said.

The study was to be presented Friday at a meeting of the American Physiological Society in Fort Lauderdale, Fla.

More information

The March of Dimes has more about cystic fibrosis.

SOURCE: American Physiological Society, news release, Nov. 3, 2006
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