Hedgehog signaling controls fibroblast activation and tissue fibrosis in systemic sclerosis

Abstract

We demonstrated that the hedgehog pathway is activated in patients with SSc. Hedgehog signaling potently stimulates the release of collagen and myofibroblast differentiation in vitro and is sufficient to induce fibrosis in vivo.

These findings identify the hedgehog cascade as a profibrotic pathway in SSc.

Keywords

• systemic sclerosis
• fibrosis
• hedgehog signaling