Inhibition of ERK/MAPK signaling as potential therapy to prevent optic pathway glioma in infants with neurofibromatosis type 1.

Pediatric low-grade gliomas (pLGGs) arise primarily at early stages of development. The molecular mechanisms of pLGG gliomagenesis are unclear, as is the progenitor cell of origin. In this issue of Developmental Cell, Jecrois et al. show that NF1-associated optic pathway gliomas originate from migrating glial progenitors that have distinct MEK/ERK dependency.