GLI-1, Effector of Canonical Hedgehog Signaling Pathway, Might not be Dysregulated and has a Neutral Effect on Survival in Patients with Myelofibrosis

Activation of Hedgehog signaling was reported to be present in murine bone-marrow transplant model of primary-myelofibrosis (PMF) and increased expression of Hedgehog target genes was reported in granulocytes isolated from unselected patients with myeloproliferative-neoplasms (MPN). However, therapeutic Hedgehog inhibition showed inconsistent effects on GLI1 (effector of hedgehog signaling) and only modest clinical results in myelofibrosis so far.