Regional identity of human neural stem cells determines oncogenic responses to histone H3.3 mutants

Research output: Contribution to journalJournal articleResearchpeer-review

Documents

  • Bardini Bressan, Raul
  • Benjamin Southgate
  • Kirsty M. Ferguson
  • Carla Blin
  • Vivien Grant
  • Neza Alfazema
  • Jimi C. Wills
  • Maria Angeles Marques-Torrejon
  • Gillian M. Morrison
  • James Ashmore
  • Faye Robertson
  • Charles A.C. Williams
  • Leanne Bradley
  • Alex von Kriegsheim
  • Richard A. Anderson
  • Simon R. Tomlinson
  • Steven M. Pollard

Point mutations within the histone H3.3 are frequent in aggressive childhood brain tumors known as pediatric high-grade gliomas (pHGGs). Intriguingly, distinct mutations arise in discrete anatomical regions: H3.3-G34R within the forebrain and H3.3-K27M preferentially within the hindbrain. The reasons for this contrasting etiology are unknown. By engineering human fetal neural stem cell cultures from distinct brain regions, we demonstrate here that cell-intrinsic regional identity provides differential responsiveness to each mutant that mirrors the origins of pHGGs. Focusing on H3.3-G34R, we find that the oncohistone supports proliferation of forebrain cells while inducing a cytostatic response in the hindbrain. Mechanistically, H3.3-G34R does not impose widespread transcriptional or epigenetic changes but instead impairs recruitment of ZMYND11, a transcriptional repressor of highly expressed genes. We therefore propose that H3.3-G34R promotes tumorigenesis by focally stabilizing the expression of key progenitor genes, thereby locking initiating forebrain cells into their pre-existing immature state.

Original languageEnglish
JournalCell Stem Cell
Volume28
Issue number5
Pages (from-to)877-893.e9
ISSN1934-5909
DOIs
Publication statusPublished - 2021
Externally publishedYes

Bibliographical note

Publisher Copyright:
© 2021 The Authors

    Research areas

  • cancer, DIPG, forebrain, glioblastoma, histone H3.3, neural stem cells, neurodevelopment, pediatric high-grade glioma, ZMYND11

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