Researchers identify potential target for treating scleroderma

"We can now think of manipulating Fibulin-5 as a therapeutic strategy to treat skin fibrosis," said Varghese.

"With further studies, we are looking to provide hope for a disease condition that contributes to approximately 30 percent of all deaths worldwide," added Jamora.

Their research reveals a hitherto unexplored mechanism responsible for the development of fibrosis in skin tissues and is potentially relevant to other organs such as the lungs, heart, kidney and liver that develop fibrosis as well.

The scar-like tissue in fibrosis cause the affected areas to stiffen, swell, and eventually lose their normal function.

The mechanisms underlying fibrosis development are poorly understood and the condition has no proven treatment or cure, but the team focused on the protein elastin and discovered that elastic fibers contributed to fibrosis in two ways - by stiffening the tissue and promoting inflammation.

Since elastin formation was known to be dependent on the molecule Fibulin 5, the researchers began to investigate its role in the disease. Through experiments in genetically engineered mice and skin tissues of human patients, they showed possible connection between elevated levels of Fibulin-5 and the abnormally large amounts of elastin formation in fibrosis.

As a next step, the researchers are exploring methods to inhibit Fibulin-5 in fibrotic tissue to arrest the spread of the condition.