Recent research suggests a possible explanation for pulmonary fibrosis, a devastating disease that is poorly understood. Investigators at Cedars-Sinai reported that in human lung tissue and in mice, sufferers of the disease appear to have faulty AEC2 cells, stem cells that repair epithelial tissue.
In people with pulmonary fibrosis, something goes wrong with AEC2 cells, the study found. Compared with lung tissue of disease-free individuals, lung tissue from patients with pulmonary fibrosis had far fewer AEC2 cells, and those that remained were less able to renew themselves. Surfaces of these cells had lower concentrations of hyaluronan, a chemical substance that promotes tissue repair and renewal. Further, in laboratory mice, the team found that by deleting this substance, they could produce the type of scarring found in pulmonary fibrosis after lung injury.
“These findings are the first published evidence that idiopathic pulmonary fibrosis is primarily a disease of AEC2 stem cell failure,” said Carol Liang, MD, assistant professor of Medicine at Cedars-Sinai and the study’s first author. “In further studies, we will explore how the loss of hyaluronan promotes fibrosis and how it might be restored to cell surfaces. These endeavors could lead to new therapeutic approaches.”
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