Inhibition of hedgehog signaling for the treatment of murine sclerodermatous chronic graft-versus-host disease

Abstract

Here, we examined the role of the hedgehog pathway in sclerodermatous cGvHD. Hedgehog signaling was activated in human and murine cGvHD.

Treatment with LDE223, a highly selective small molecule antagonist of the hedgehog coreceptor Smoothened (Smo), abrogated the activation of hedgehog signaling and protected against experimental cGvHD.

Keywords

• systemic sclerosis
• fibrosis
• sclerodermatous GvHD
• LDE223