Epigenetic dysregulation in brain tumors and neurodevelopment

M. M. Hefti, N. Tsankova

Research output: Chapter in Book/Report/Conference proceedingChapterpeer-review

Abstract

Epigenetics plays a well-known role in the regulation of stem cell identity, including the specification of cell lineages within the central nervous system, through a complex, regulated interplay between DNA methylation and the remodeling of chromatin. More recently, dysregulation of these mechanisms has been implicated in the pathobiology of brain tumors. Mutations in genes, such as H3F3A, ATRX, IDH, INI-1, and others cause global dysregulation of epigenetic signaling that leads to abnormal expression of oncogenes and inhibition of tumor suppressors, leading to disordered cell growth. Intriguingly, mutations in some of the same chromatin remodeling genes can lead to neurodevelopmental disorders or to brain tumors, depending on whether they are germline or somatic in origin. This has impelled new interest in understanding better the role of stem cells and various neural progenitors as putative tumor cells of origin in specific niches within the central nervous system.

Original languageEnglish
Title of host publicationNeuropsychiatric Disorders and Epigenetics
PublisherElsevier Inc.
Pages261-276
Number of pages16
ISBN (Print)9780128002261
DOIs
StatePublished - 2017

Keywords

  • ATRX
  • Chromatin
  • Epigenetics
  • Glioblastoma
  • Glioma
  • Histone H3
  • IDH
  • Medulloblastoma
  • Methylation
  • Neuropathology

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