Cedars-Sinai Given $12M to Study IPF, Chronic Lung Allograft Dysfunction

The National Institutes of Health (NIH) has granted $12 million to researchers at Cedars-Sinai Medical Center to continue studying two lung diseases — idiopathic pulmonary fibrosis and chronic lung allograft dysfunction.

Idiopathic pulmonary fibrosis (IPF) is characterized by progressive scarring of lung tissue which leads to difficulty breathing. Chronic lung allograft dysfunction is a complication that causes lung transplants to fail, often leading to death.

As both diseases are not well understood at this time, physicians are able to only slow disease progression.

“Doctors can treat some symptoms and even slow the disease process in certain cases, but lack of knowledge keeps us from developing cures,” Paul Noble, MD, director of the Women’s Guild Lung Institute and chair of the Department of Medicine at Cedars-Sinai, said in a news release from the medical center. “That is why research is so critical.”

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The award was granted to Noble and his colleagues to build on previous groundbreaking research, which was also funded by the NIH.

Noble’s research is focused on fibrosis, which refers to the growth of fibrous, connective tissue that is involved in the development of both of these lung disorders.

In previous studies, Noble’s team identified the key cell types that drive IPF. They also found two drugs that slowed disease progression and discovered some of the molecular mechanisms that cause fibrosis.

This grant will be used to fund three projects that will build on this previous research.

The first project relates to immune checkpoint inhibitors, which are highly expressed proteins in the fibrous connective tissue of patients with IPF or those that have undergone lung transplants. Immune checkpoint inhibitors are also secreted by cancer cells to prevent immune cells from finding them.

“These tissue cells are behaving like cancer cells, raising the possibility that existing anti-cancer drugs may be useful in treating these conditions,” Noble added.

The second project, which will be led by Barry Stripp, PhD, will investigate a cell type known as epithelial progenitor cells in idiopathic pulmonary fibrosis. Prior studies have shown that these cells, which maintain the lung’s airways and air sacs and allow gas exchange during breathing, are defective in patients with IPF.

The project will investigate the role of signaling by the p53 protein, which is known to regulate the activity of these progenitor cells.

The third project, which will be led by John Belperio, MD, will investigate the same cell type — epithelial progenitor cells — in chronic lung allograft dysfunction. Researchers hypothesize that the lung’s reserves of epithelial progenitor cells can influence how the body responds to the trauma of transplantation.

“This prestigious National Institutes of Health grant recognizes the investigators’ significant progress in uncovering the causes of two mysterious, destructive lung conditions,” said Shlomo Melmed, MB, ChB, executive vice president and dean of the Faculty at Cedars-Sinai.

“The new funding will help Cedars-Sinai, an internationally recognized leader in lung fibrosis research, continue to expand the frontiers of knowledge in pulmonary medicine.”

This grant is supported by the National Heart, Lung, and Blood Institute of the NIH under award number P01HL108793.