A four-year, $2.4 million federal grant given investigators at Boston University School of Medicine (BUSM) will support work to create a 3D cell model system helping to explain the processes that initiate and drive the progression of idiopathic pulmonary fibrosis (IPF).
According to a press release, the project will be led by Darrell Kotton, MD, the David C. Seldin professor of medicine at BUSM, with funding from the National Heart, Lung, and Blood Institute, part of the National Institutes of Health.
The biological mechanisms that drive pulmonary fibrosis, characterized by scarring (fibrosis) in the lungs, are not completely understood, hampering the development of therapies.
Cell models are a useful tool for researchers seeking to better understand disease mechanisms, and the BUSM team plans to develop a human 3D model system for IPF, whose underlying cause of scarring is not known. Familial pulmonary fibrosis, in contrast, refers to lung scarring that runs in families.
To do this, researchers will use induced pluripotent stem cells (iPSCs) generated from individuals with IPF or familial pulmonary fibrosis. iPSCs are a type of stem cell, meaning they are able to differentiate into other types of cells. “Induced” means that these stem cells have been reverse-engineered from other cell types, typically skin cells.
Researchers will use these iPSCs to generate lung cells for their models. The team specifically intends to investigate the role of epithelial cells, which line the airways of the lungs, in IPF. A growing body of research has indicated that these cells play an integral role in the development of scarring in the lungs.
Studies have also indicated that lung epithelial cells have unusually short telomeres, structures at the ends of chromosomes that help to protect genetic material from damage. However, the consequences of these and other genetic changes to the cells, as they relate to IPF, is not clear, in part because specialized cell models would be needed for such studies.
“Without access to patient-specific human [cell] model systems, there are limited options for testing hypotheses of how epithelial changes induced by [genetic variations] or telomerase perturbations might mechanistically contribute to IPF,” Kotton said in the release.
According to Kotton, this project will provide insight into IPF development and might lead to new treatments.
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