The Hedgehog (Hh) pathway is a highly conserved signaling system regulating a range of developmental processes. A 1997 paper by Goodrich and colleagues provided major contributions to understanding the Hh pathway by mutating the gene encoding the Hh receptor, Patched, and thereby developing a mouse model for a human cancer predisposition syndrome, known as Gorlin syndrome. These studies provided one of the first genetically engineered mouse models for brain tumors.
Keywords: Gorlin syndrome; Patched; Shh; basal cell carcinoma; medulloblastoma; pediatric brain tumor.
Copyright © 2018 Elsevier Ltd. All rights reserved.