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Thomas Jefferson University Research Magazine

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Abstract

Up to 40 percent of disease mortality in developed countries results from fibrotic diseases, a family of conditions where the build-up of scarlike tissue interferes with the body’s normal function. These conditions can be systemic (such as systemic sclerosis and scleroderma) or affect individual organs such as the lung, liver, kidney, heart or eye. Because the mechanisms underlying these conditions are poorly understood and treatments are few, Jefferson has made a major commitment to basic and translational research on a broad range of fibrotic diseases.

Pulmonary Fibrosis (PF) causes progressive, potentially fatal scarring of the lung. PF results, in part, from the inability of aging lung epithelial cells to regenerate after injury. This may be because aging cells do not direct nutrients to areas at greatest need after injury. Ross Summer, MD, professor of medicine, has been working to determine the interconnections between cellular metabolism and injury repair. His studies on young cells’ metabolic adaptation to injury offer clues on how aging compromises the repair process—and suggests therapeutic approaches. For example, Dr. Summer has found that blocking lipid synthesis, alone, can cause fibrotic scarring in animal models; and, in counterpoint, has demonstrated that increased lipid production could reduce fibrosis-caused lung scarring significantly. He is now working to identify and test molecules that could have therapeutic effect by enhancing or protecting lipid synthesis.

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