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Proteins in lungs of patients with cystic fibrosis

und in different areas, researchers speculate that they may have slightly different functions and may fight different infectious agents.

Investigation with cystic fibrosis

People with cystic fibrosis have a faulty gene, which normally controls the movement of salt into and out of cells and this controls the movement of water, too, Bingle explained. In a patient with cystic fibrosis, there is too little salt and water on the outside of cells lining the airways. This means that the normally thin protective layer of mucus becomes thick and very difficult to move. It is very difficult for the patient to cough up the thick mucus, so the airways get clogged. The trapped mucous becomes a haven for infectious agents, which leads to long-term infection, inflammation and scarring. Most patients will eventually need lung transplants in order to survive, she said.

In this study, the researchers compared the tissue of 21 cystic fibrosis patients -- all of whom had end stage disease and were scheduled for a transplant -- to healthy tissue. The healthy tissue was obtained from 10 patients undergoing surgical removal of a lung tumor. The researchers used the healthy portion of lung tissue which is usually excised along with the tumor.

The study looked at tissue samples from the lung's upper airway, just below the trachea, and from lower down in the airway, in the peripheral lung, where gas exchange takes place.

This lower region of the lung has small airways as well as the gas-exchange tissue. They used a staining technique to find SPLUNC1, SPLUNC2 and LPLUNC1.

In normal lungs: · SPLUNC1 is found predominantly in the upper airways, rarely in the smaller airways and is absent in the gas-exchange tissue of the peripheral lung. · LPLUNC1 is found in both the small airways and in the upper airways · SPLUNC2 is found in the mouth, but not in the lungs The study found that the presence of SPLUNC1 is "massively i
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