Grant: Project Grant - PA: Myalgic Encephalomyelitis and IMHA Mandate Areas

Fibrotic diseases such as scleroderma have no therapy. These diseases are characterized by extensive scar tissue deposition. The cells (fibroblasts) within the scar are subject to enhanced tension due to the surrounding scar tissue. They also themselves are abnormal in that they exert excessive tension on the scar tissue. It is believed that this excessive tension is responsible for the maintenance of the scar. A protein called CCN2 is expressed specifically in scar tissue and promotes this tension. A related protein CCN3 is not found in scar tissue, but normally acts to inhibit CCN2. We want to test whether CCN3 and peptides derived from CCN3) can block scleroderma fibrosis. Moreover, we want to test if monitoring the CCN2: CCN3 ratio in patients can be used to monitor disease progression At the end of the funding period, we will design and initiate clinical trials with our peptides to see if we can block fibrosis clinically.