Nanog-driven cell-reprogramming and self-renewal maintenance in Ptch1 ^+/- granule cell precursors after radiation injury.

ABSTRACT: Medulloblastoma (MB) is the most common pediatric brain tumor, comprising four distinct molecular variants, one of which characterized by activation of the Sonic Hedgehog (SHH) pathway, driving 25-30% of sporadic MB. SHH-dependent MBs arise from granule cell precursors (GCPs), are fatal in 40-70% of cases and radioresistance strongly contributes to poor prognosis and tumor recurrence. Patched1 heterozygous (Ptch1 ^+/-) mice, carrying a germ-line heterozygous inactivating mutation in the Ptch1 gene, the Shh receptor and negative regulator of the pathway, are uniquely susceptible to MB development after radiation damage in neonatal cerebellum. Here, we irradiated ex-vivo GCPs isolated from cerebella of neonatal WT and Ptch1 ^+/- mice. Our results highlight a less differen

SUBMITTER: Tanno B 

PROVIDER: S-EPMC5660207 | biostudies-literature | 2017 Oct

REPOSITORIES: biostudies-literature