Nunzia Caporarello, Ph.D.

Institution: Mayo Clinic

Project: Targeting vascular rarefaction and loss of endothelial identity in pulmonary fibrosis

Grant(s): Dalsemer Grant

Idiopathic pulmonary fibrosis is a chronic lung disease that causes scarring of the lungs, leading to organ failure and death. Therapies to treat this disease are limited, making further studies on mechanisms responsible for the disease a priority. Although pulmonary blood vessels are abundant in the lungs and contribute to regeneration and repair of adult organs, including lungs, their role in pulmonary fibrosis is mostly unknown. We will investigate the role of ERG, a gene which controls the function and the biology of the endothelial cells (the cells forming the blood vessels), in regulating the development of pulmonary fibrosis. We will define ERG’s role as a potential therapeutic target which will restore the stability of endothelial cells and promote resolution of the disease.

Update: Over the past year we have investigated the role of ERG in regulating endothelial repair after injury of the lungs. We have discovered that lack of ERG in the pulmonary vasculature of mice challenged with bleomycin (a chemical agent used in research to induce fibrosis in animal models) promoted persistent lung fibrosis. Based on these findings, we are now testing whether ERG can be targeted to serve as an effective therapy to slow progression of lung fibrosis.

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