BALTIMORE, March 28 (UPI) -- Genes triggering idiopathic pulmonary fibrosis (IPF), a fatal lung disease, have been found by a group at Baltimore-based Johns Hopkins Cancer Center.
Mary Armanios led the team that discovered that the inherited form of the disorder, which scars the lungs and is often fatal within three years, could be traced to two genes.
These genes regulate telomerase, an enzyme that keeps the protective structures at the ends of chromosomes -- called telomeres -- long enough to keep the chromosome's ends from fraying and triggering cell death.
When the researchers screened the DNA in blood samples from 73 people with IPF, they found the two genetic mutations in 8 percent of them.
People with the mutations had telomeres that were one-third of the length of those in family members who did not carry the defect.
The researchers said it is also possible that patients with non-inherited IPF may also have short telomeres, possibly caused by aging or smoking, which are the two main risk factors for the disease.
If further research proves them right, it may change the way IPF is treated, Armanios said.
The research appears in the March 29 issue of the New England Journal of Medicine.
