Two federal grants totaling $6.1 million were awarded to Vanderbilt University Medical Center (VUMC) and the Translational Genomics Research Institute (TGen) to investigate the molecular origin of idiopathic pulmonary fibrosis (IPF), as well as of other forms of pulmonary fibrosis (PF).
The first is a $3.5-million five-year grant awarded by the National Institutes of Health’s National Heart, Lung and Blood Institute to study the genetic causes of IPF. The other grant, of $2.6 million, was given by the U.S. Department of Defense (DOD) to study other genetic factors associated with non-IPF forms of pulmonary fibrosis.
In the first project, researchers from VUMC and TGen will work together to explore the molecular changes that happen in the lungs during IPF. The goal is to answer fundamental questions about what drives the disease.
Researchers will use single-cell sequencing, an advanced technology, to closely profile the activity of genes in individual cells within the lungs of IPF patients versus healthy individuals. Rather than looking at a blend of cells from a piece of tissue, this type of technology measures the activity of a wide number of genes in individual cells.
The scientists hope that this strategy will help them to spot more precisely the changes in gene activity specific to the lungs of IPF patients, which may offer clues about the molecular origin of the disease. This could also result in finding an IPF molecular signature that may help provide an earlier diagnosis, and possibly develop better treatments for patients.
“This is exciting because prior studies of using lung tissue from IPF patients could not tell us what the individual cells are doing to cause this disease. This study is trying to precisely understand how all of the different types of cells in the lung behave differently compared to normal lungs,” Jonathan Kropski, MD, assistant professor at VUMC and one of the principal investigators of the study, said in a VUMC release written by Nancy Humphrey.
“By improving our understanding of the critical molecular drivers of IPF, we are very hopeful that these studies will set us on a path to more precise and more effective treatments that improve the lives for the hundreds of thousands of patients with this disease,” Kropski added.
IPF affects an estimated 50,000–100,000 people per year in the U.S. The cause of the disease remains unknown, with the best treatment available being lung transplant. But the procedure carries risks, and not all patients are suitable candidates. The other IPF therapies slow disease progression or relieve symptoms.
The second grant will fund research about other forms of pulmonary fibrosis. The study will complement the first one by closely investigating the mechanisms that lead to lung scarring and stiffening in other forms of PF.
“Together both grants will help us to closely examine all lung tissue and search for the genomic factors that drive all pulmonary fibrosis,” Nicholas Banovich, PhD, assistant professor at TGen’s and the other study leader, said in a press release.
“Since IPF represents about 20 percent of all lung fibrosis, the second grant from DOD will help allow us to characterize the many different genomic drivers of the other 80 percent of patients with lung fibrosis, potentially giving us a more universal assessment of lung disease and how best to treat these debilitating conditions,” Banovich said.