A therapeutic window for preventive therapy in NF1-associated optic pathway glioma

Abstract

Pediatric low-grade gliomas (pLGGs) are almost universally driven by abnormal activation of RAS-mediated MEK-ERK/MAPK signaling pathway. pLGGs predominantly occur in children, suggesting that they originate in an ERK-dependent neural stem/progenitor population(s) transiently present in the developing brain. Our recent preclinical study reveals a cell-lineage-of-origin and develops a chemopreventative therapeutic strategy.

Keywords

• neurofibromatosis type 1
• pediatric low-grade glioma
• neural stem cells
• hypothalamic ventricular zone (hvz)
• radial glia (rg)