Mucus-related gene tied to lung disease
Pulmonary fibrosis associated with high production of protein
By Nathan Seppa
Carrying a variant form of a gene might add to a person’s risk of developing a devastating lung disease called pulmonary fibrosis. The variant induces the body to overproduce mucus in the lungs, researchers report in the April 21 New England Journal of Medicine.
Pulmonary fibrosis, a scarring of the lungs, is a rare but lethal condition that currently affects about 100,000 people in the United States. Although the cause of pulmonary fibrosis is unclear, scientists suspect a combination of genetic factors and environmental irritants such as exposure to cigarette smoke, asbestos fibers or silica dust.
There is no cure, and survival after diagnosis is often only three to five years, says James Kiley, a pulmonary physiologist at the National Heart, Lung and Blood Institute in Bethesda, Md., who wasn’t part of the new study. The findings might point to one of the root causes of pulmonary fibrosis, he says, “and may lead to something to prevent or manage this disease a bit better.”
Mucus protects air passages from foreign pathogens and pollutants. But too much mucus in the lungs can become sticky and difficult to clear, increasing susceptibility to infection and chronic inflammation. The delicate balance between maintaining a protective mucus coating and mucus overproduction is upset in certain lung diseases.