@article{52355f0cb1644b3d98836439b005fbcb,
title = "Epigenetic reprogramming and chromatin accessibility in pediatric diffuse intrinsic pontine gliomas: A neural developmental disease",
abstract = "Diffuse intrinsic pontine glioma (DIPG) is a rare but deadly pediatric brainstem tumor. To date, there is no effective therapy for DIPG. Transcriptomic analyses have revealed DIPGs have a distinct profile from other pediatric high-grade gliomas occurring in the cerebral hemispheres. These unique genomic characteristics coupled with the younger median age group suggest that DIPG has a developmental origin. The most frequent mutation in DIPG is a lysine to methionine (K27M) mutation that occurs on H3F3A and HIST1H3B/C, genes encoding histone variants. The K27M mutation disrupts methylation by polycomb repressive complex 2 on histone H3 at lysine 27, leading to global hypomethylation. Histone 3 lysine 27 trimethylation is an important developmental regulator controlling gene expression. This review discusses the developmental and epigenetic mechanisms driving disease progression in DIPG, as well as the profound therapeutic implications of epigenetic programming.",
keywords = "DIPG, H3 K27M, epigenetics, histone variants",
author = "Mendez, {Flor M.} and N{\'u}{\~n}ez, {Felipe J.} and Garcia-Fabiani, {Maria B.} and Santiago Haase and Stephen Carney and Gauss, {Jessica C.} and Becher, {Oren J.} and Lowenstein, {Pedro R.} and Castro, {Maria G.}",
note = "Funding Information: This work was supported by National Institutes of Health/ National Institute of Neurological Disorders & Stroke (NIH/ NINDS) Grants R37-NS094804, R01-NS105556, R21-NS107894 to M.G.C.; NIH/NINDS Grants R01-NS076991, R01-NS082311, and R01-NS096756 to P.R.L.; NIH/NIBIB R01-EB022563 and NCI/UO1-CA-224160 to M.G.C. and P.R.L.; the Department of Neurosurgery; Leah{\textquoteright}s Happy Hearts Foundation, ChadTough Foundation, Pediatric Brain Tumor Foundation, and Smiles for Sophie Forever Foundation to M.G.C. and P.R.L. RNA Biomedicine Grant F046166 to M.G.C. NIH/NINDS-F31NS103500 and Rackham Pre-doctoral Fellowship to F.M.M; S.C. is supported by NIH/NCI-T32-CA009676. OJB is supported by R01-CA197313, American Cancer Society RSG-16-218-01-TBG, the Rory David Deutsch Foundation, Max Cure Foundation, Cancer Smashers, and Madox{\textquoteright}s Warriors. Funding Information: This work was supported by National Institutes of Health/ National Institute of Neurological Disorders & Stroke (NIH/ NINDS) Grants R37-NS094804, R01-NS105556, R21-NS107894 to M.G.C.; NIH/NINDS Grants R01-NS076991, R01-NS082311, and R01-NS096756 to P.R.L.; NIH/NIBIB R01-EB022563 and NCI/UO1-CA-224160 to M.G.C. and P.R.L.; the Department of Neurosurgery; Leah's Happy Hearts Foundation, ChadTough Foundation, Pediatric Brain Tumor Foundation, and Smiles for Sophie Forever Foundation to M.G.C. and P.R.L. RNA Biomedicine Grant F046166 to M.G.C. NIH/NINDS-F31NS103500 and Rackham Pre-doctoral Fellowship to F.M.M; S.C. is supported by NIH/NCI-T32-CA009676. OJB is supported by R01-CA197313, American Cancer Society RSG-16-218-01-TBG, the Rory David Deutsch Foundation, Max Cure Foundation, Cancer Smashers, and Madox's Warriors. Publisher Copyright: {\textcopyright} 2019 The Author(s). All rights reserved.",
year = "2020",
month = feb,
day = "20",
doi = "10.1093/neuonc/noz218",
language = "English (US)",
volume = "22",
pages = "195--206",
journal = "Neuro-Oncology",
issn = "1522-8517",
publisher = "Oxford University Press",
number = "2",
}
