Dr. Riches and his group have previously shown that fibroblasts normally die through apoptosis following stimulation of a receptor called Fas. Furthermore they showed that these cells will not die when Fas becomes associated with an inhibitory protein, the PTPN13 molecule. They were able to demonstrate that by blocking the association between Fas and PTPN13, fibroblast cells are once again able to undergo Fas-receptor induced death. These findings suggest that the development of a compound that blocks the Fas/PTPN13 interaction could serve as a therapeutic modality to treat IPF.

Investigators are currently screening libraries to identify molecules that will interfere in the binding of PTPN13 to Fas.

Therapeutic uses for treatment of idiopathic pulmonary fibrosis. Other fibrotic conditions are being explored.

Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressive fibrosing interstitial pneumonia, primarily occurring in older adults. It is caused by injury and aberrant repair of the lower lung resulting in accumulation of fibroblasts. These cells produce abundant amounts of collagen and contribute to the formation of scar tissue. In normal wound repair, fibroblasts die and are removed at the completion of the repair process. In IPF, fibroblasts persist and accumulate in the lung, leading to progressive fibrosis, dyspnea, hypoxemia, and death within 5 years of diagnosis.