Researchers identify potential target for treating scleroderma

An international team of scientists has identified a new molecule involved in life-threatening scleroderma or skin fibrosis, paving the way for designing therapies for the disease.

The new study is the first to investigate the role of this molecule in skin fibrosis, a disease characterized by the inflammation and hardening of skin tissue, which robs healthy skin of its softness and pliability and makes it tough, scaly and rough instead.

Scientists have now discovered a new player involved in the development of skin fibrosis: a molecule called Fibulin-5, said a statement from inStem (Institute for Stem Cell Biology and Regenerative Medicine), National Centre for Biological Sciences in Bengaluru on Friday.

The team, led by Colin Jamora, a biologist at the IFOM-inSTEM Joint Research Laboratory in India, and bioengineering professor Shyni Varghese at the University of California, San Diego published their findings on October 15 in the issue of Nature Communications.

"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.