Epigenetic mechanisms are essential to regulate gene expression during normal development. However, they are often disrupted in pathological conditions including tumours, where they contribute to their formation and maintenance through altered gene expression. In recent years, next generation genomic techniques has allowed a remarkable advancement of our knowledge of the genetic and molecular landscape of paediatric brain tumours and have highlighted epigenetic deregulation as a common hallmark in their pathogenesis. This review describes the main epigenetic dysregulations found in paediatric brain tumours, including at DNA methylation and histone modifications level, in the activity of chromatin-modifying enzymes and in the expression of non-coding RNAs. How these altered processes influence tumour biology and how they can be leveraged to dissect the molecular heterogeneity of these tumours and contribute to their classification is also addressed. Finally, the availability and value of preclinical models as well as the current clinical trials exploring targeting key epigenetic mediators in paediatric brain tumours are discussed.
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February 2022
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Cover Image
Cover Image
The highly conserved enzyme IMPDH plays an essential role in purine biosynthesis and is tightly regulated by many different mechanisms. Depicted here are cryo-EM structures of the large retinal splice variant of IMPDH1 in different filament assembly conformations overlaid on a cryo-EM micrograph of IMPDH1 filaments. Cover artwork created by Jesse Hansen.
Review Article|
January 25 2022
Epigenetic mechanisms in paediatric brain tumours: regulators lose control
Sara Badodi;
Blizard Institute, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, 4 Newark Street, London E1 2AT, U.K.
Correspondence: Sara Badodi (s.badodi@qmul.ac.uk) or Silvia Marino (s.marino@qmul.ac.uk)
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Silvia Marino
Blizard Institute, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, 4 Newark Street, London E1 2AT, U.K.
Correspondence: Sara Badodi (s.badodi@qmul.ac.uk) or Silvia Marino (s.marino@qmul.ac.uk)
Search for other works by this author on:
Publisher: Portland Press Ltd
Received:
September 15 2021
Revision Received:
November 28 2021
Accepted:
December 23 2021
Online ISSN: 1470-8752
Print ISSN: 0300-5127
© 2022 The Author(s). Published by Portland Press Limited on behalf of the Biochemical Society
2022
Biochem Soc Trans (2022) 50 (1): 167–185.
Article history
Received:
September 15 2021
Revision Received:
November 28 2021
Accepted:
December 23 2021
Citation
Sara Badodi, Silvia Marino; Epigenetic mechanisms in paediatric brain tumours: regulators lose control. Biochem Soc Trans 28 February 2022; 50 (1): 167–185. doi: https://doi.org/10.1042/BST20201227
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