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Researchers Prevent And Reverse Fibrosis In A Mouse Model Of Stiff Skin Syndrome; Study Shows Promise For Scleroderma

SAN FRANCISCO, Oct. 9, 2013 /PRNewswire-USNewswire/ -- The Scleroderma Research Foundation today said that researchers at The Johns Hopkins University working in a novel mouse model of Stiff Skin Syndrome have made key discoveries that may have broad implications for future scleroderma therapy. In a report to be published online on October 10th in Nature, scientists show that integrin-modulating agents can arrest fibrosis in a model of scleroderma-like skin fibrosis and, more strikingly, that established fibrosis can be resolved with such agents. Although years away from an approved drug, the conclusion stands in sharp contrast to traditional thinking about scleroderma's irreversible advance and its resistance to therapeutic intervention. The Scleroderma Research Foundation has supported the work for the past six years. 

Systemic sclerosis (commonly referred to as scleroderma) is a rare autoimmune disease affecting approximately 1 in 4,000 Americans. A signature symptom is fibrosis of the skin, although the disease typically affects the lungs, kidneys and other organs with life-threatening consequences. Like many complex, adult-onset  autoimmune diseases, animal models replicate some, but not all features of the disease. This gap has slowed work in the scleroderma field. 

In the Nature report, a group led by Hal Dietz, M.D., focused on a genetic fibrotic disease called Stiff Skin Syndrome to provide insights. Dr. Dietz is a Professor of Genetics at The Johns Hopkins University School of Medicine, Director of the William S. Smilow Center for Marfan Syndrome Research and an Investigator for the Howard Hughes Medical Institute. "We looked to Stiff Skin Syndrome, an ultra-rare form of inherited scleroderma-like skin fibrosis as a means to understand the very similar skin fibrosis of systemic sclerosis," explains Dietz. "Reasoning that if we could understand how a single point mutation in the fibrillin-1 (FBN1) protein causes skin fibrosis in Stiff Skin Syndrome (SSS), this would help us to understand the biological pathways involved in systemic sclerosis, a typically acquired, adult-onset autoimmune disease." 

"There may be a final common pathway by which cells lose their normal control," postulates Dietz, "and even if one condition is genetic and the other is not, they may both funnel down to the same mechanism."

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