References

The 2021 WHO classification of tumors of the central nervous system: a summary

10.1093/neuonc/noab106 · 2021 · External reference

Inhibition of PRC2 activity by a gain-of-function H3 mutation found in pediatric glioblastoma

10.1126/science.1232245 · 2013 · External reference

EZH2 is a potential therapeutic target for H3K27M-mutant pediatric gliomas

10.1038/nm.4293 · 2017 · External reference

K27M mutation in histone H3.3 defines clinically and biologically distinct subgroups of pediatric diffuse intrinsic pontine gliomas

10.1007/s00401-012-0998-0 · 2012 · External reference

H3 K27M-altered glioma and diffuse intrinsic pontine glioma: semi-systematic review of treatment landscape and future directions

10.1093/neuonc/noad220 · 2024 · External reference

Prognostic indicators for H3K27M-Mutant diffuse midline glioma: a population-based retrospective surveillance, epidemiology, and end results database analysis

10.1016/j.wneu.2023.07.001 · 2023 · External reference

Epigenetic protein families: a new frontier for drug discovery

10.1038/nrd3674 · 2012 · External reference

The expanding landscape of ’oncohistone’ mutations in human cancers

10.1038/s41586-019-1038-1 · 2019 · External reference

Mutational landscape of uterine and ovarian carcinosarcomas implicates histone genes in epithelial-mesenchymal transition

10.1073/pnas.1614120113 · 2016 · External reference

Histone mutations in cancer

10.1042/bst20210567 · 2023 · External reference

The histone H3.3K36M mutation reprograms the epigenome of chondroblastomas

10.1126/science.aae0065 · 2016 · External reference

Chromatin structure: a repeating unit of histones and DNA

10.1126/science.184.4139.868 · 1974 · External reference

Nucleosome structure and function

10.1021/cr500373h · 2015 · External reference

Crystal structure of the nucleosome core particle at 2.8 A resolution

10.1038/38444 · 1997 · External reference

Translating the histone code

10.1126/science.1063127 · 2001 · External reference

Regulation of chromatin by histone modifications

10.1038/cr.2011.22 · 2011 · External reference

Chromatin modifications and their function

10.1016/j.cell.2007.02.005 · 2007 · External reference

Histone methylation: a dynamic mark in health, disease and inheritance

10.1038/nrg3173 · 2012 · External reference

Histone lysine methylation dynamics: establishment, regulation, and biological impact

10.1016/j.molcel.2012.11.006 · 2012 · External reference

Histone lysine demethylases: emerging roles in development, physiology and disease

10.1038/nrg2218 · 2007 · External reference

KDM1 histone lysine demethylases as targets for treatments of oncological and neurodegenerative disease

10.2217/epi.15.9 · 2015 · External reference

50 years of protein acetylation: from gene regulation to epigenetics, metabolism and beyond

10.1038/nrm3931 · 2015 · External reference

Perceiving the epigenetic landscape through histone readers

10.1038/nsmb.2436 · 2012 · External reference

Readers of histone modifications

10.1038/cr.2011.42 · 2011 · External reference

Targeting bromodomains: epigenetic readers of lysine acetylation

10.1038/nrd4286 · 2014 · External reference

The mechanisms behind the therapeutic activity of BET bromodomain inhibition

10.1016/j.molcel.2014.05.016 · 2014 · External reference

Driver mutations in histone H3.3 and chromatin remodelling genes in paediatric glioblastoma

10.1038/nature10833 · 2012 · External reference

Somatic histone H3 alterations in pediatric diffuse intrinsic pontine gliomas and non-brainstem glioblastomas

10.1038/ng.1102 · 2012 · External reference

Therapeutic targeting of polycomb and BET bromodomain proteins in diffuse intrinsic pontine gliomas

10.1038/nm.4296 · 2017 · External reference

Multiple modes of PRC2 inhibition elicit global chromatin alterations in H3K27M pediatric glioma

10.1126/sciadv.aau5935 · 2018 · External reference

Reduced H3K27me3 and DNA hypomethylation are major drivers of gene expression in K27M mutant pediatric high-grade gliomas

10.1016/j.ccr.2013.10.006 · 2013 · External reference

Pervasive H3K27 acetylation leads to ERV expression and a therapeutic vulnerability in H3K27M gliomas

10.1016/j.ccell.2019.04.004 · 2019 · External reference

BET bromodomain inhibition potentiates radiosensitivity in models of H3K27-altered diffuse midline glioma

10.1172/jci174794 · 2024 · External reference

Systematic review of diffuse hemispheric glioma, H3 G34-mutant: outcomes and associated clinical factors

2022 · External reference

A review of diffuse hemispheric glioma, H3 G34-mutant disease development, DNA repair, microenvironment, and treatments on the horizon

10.1038/s41698-025-01070-w · 2025 · External reference

Glioblastoma with primitive neuronal component: an immunohistochemical study and review of literature

10.1016/j.jocn.2021.09.004 · 2021 · External reference

Mutations in SETD2 and genes affecting histone H3K36 methylation target hemispheric high-grade gliomas

10.1007/s00401-013-1095-8 · 2013 · External reference

Effects of H3.3G34V mutation on genomic H3K36 and H3K27 methylation patterns in isogenic pediatric glioma cells

10.1186/s40478-020-01092-4 · 2020 · External reference

H3.3-G34 mutations impair DNA repair and promote cGAS/STING-mediated immune responses in pediatric high-grade glioma models

10.1172/jci154229 · 2022 · External reference

Histone deacetylases and their inhibitors in cancer, neurological diseases and immune disorders

10.1038/nrd4360 · 2014 · External reference

Anticancer activities of histone deacetylase inhibitors

10.1038/nrd2133 · 2006 · External reference

Histone deacetylases and cancer: causes and therapies

10.1038/35106079 · 2001 · External reference

Functionally defined therapeutic targets in diffuse intrinsic pontine glioma

10.1038/nm.3855 · 2015 · External reference

Phase I trial of panobinostat in children with diffuse intrinsic pontine glioma: a report from the Pediatric Brain Tumor Consortium (PBTC-047)

10.1093/neuonc/noad141 · 2023 · External reference

Pre-Clinical study of panobinostat in xenograft and genetically engineered murine diffuse intrinsic pontine glioma models

10.1371/journal.pone.0169485 · 2017 · External reference

The distribution, clearance, and brainstem toxicity of panobinostat administered by convection-enhanced delivery

10.3171/2018.2.peds17663 · 2018 · External reference

PNOC015: repeated convection-enhanced delivery of MTX110 (aqueous panobinostat) in children with newly diagnosed diffuse intrinsic pontine glioma

10.1093/neuonc/noad105 · 2023 · External reference

Pediatric phase I trial and pharmacokinetic study of vorinostat: a children’s oncology group phase I consortium report

10.1200/jco.2009.25.9119 · 2010 · External reference

Phase I/II trial of vorinostat and radiation and maintenance vorinostat in children with diffuse intrinsic pontine glioma: a children’s oncology group report

10.1093/neuonc/noab188 · 2022 · External reference

Microtubule-targeting combined with HDAC inhibition is a novel therapeutic strategy for diffuse intrinsic pontine gliomas

10.1158/1535-7163.mct-23-0179 · 2023 · External reference

Dual targeting of histone deacetylases and MYC as potential treatment strategy for H3-K27M pediatric gliomas

10.7554/elife.96257.3 · 2024 · External reference

Phase I study of vorinostat and temsirolimus in newly diagnosed or progressive diffuse intrinsic pontine glioma

10.1002/pbc.31619 · 2025 · External reference

Cancer network disruption by a single molecule inhibitor targeting both histone deacetylase activity and phosphatidylinositol 3-kinase signaling

10.1158/1078-0432.ccr-12-0055 · 2012 · External reference

Dual HDAC and PI3K inhibition abrogates NFκB- and FOXM1-Mediated DNA damage response to radiosensitize pediatric high-grade gliomas

10.1158/0008-5472.can-17-3691 · 2018 · External reference

A phase 1 trial of fimepinostat in children and adolescents with relapsed and refractory solid and CNS tumors

10.1002/cam4.71417 · 2025 · External reference

Fimepinostat impairs NF-κB and PI3K/AKT signaling and enhances gemcitabine efficacy in H3.3K27M-Diffuse intrinsic pontine glioma

10.1158/0008-5472.can-23-0394 · 2024 · External reference

Optimal therapeutic targeting by HDAC inhibition in biopsy-derived treatment-naïve diffuse midline glioma models

10.1093/neuonc/noaa249 · 2021 · External reference

A phase I study of quisinostat (JNJ-26481585), an oral hydroxamate histone deacetylase inhibitor with evidence of target modulation and antitumor activity, in patients with advanced solid tumors

10.1158/1078-0432.ccr-13-0312 · 2013 · External reference

Phase II multicentre trial of oral quisinostat, a histone deacetylase inhibitor, in patients with previously treated stage IB-IVA mycosis fungoides/Sézary syndrome

10.1111/bjd.14427 · 2016 · External reference

Quisinostat, bortezomib, and dexamethasone combination therapy for relapsed multiple myeloma

10.3109/10428194.2015.1117611 · 2016 · External reference

GSK-J4: an H3K27 histone demethylase inhibitor, as a potential anti-cancer agent

10.1002/ijc.34559 · 2023 · External reference

Pharmacologic inhibition of histone demethylation as a therapy for pediatric brainstem glioma

10.1038/nm.3716 · 2014 · External reference

Radiosensitization by histone H3 demethylase inhibition in diffuse intrinsic pontine glioma

10.1158/1078-0432.ccr-18-3890 · 2019 · External reference

Tazemetostat: first approval

10.1007/s40265-020-01288-x · 2020 · External reference

Tazemetostat in advanced epithelioid sarcoma with loss of INI1/SMARCB1: an international, open-label, phase 2 basket study

10.1016/s1470-2045(20)30451-4 · 2020 · External reference

Tazemetostat for patients with relapsed or refractory follicular lymphoma: an open-label, single-arm, multicentre, phase 2 trial

10.1016/s1470-2045(20)30441-1 · 2020 · External reference

Potential of enhancer of zeste homolog 2 inhibitors for the treatment of SWI/SNF mutant cancers and tumor microenvironment modulation

10.1002/ddr.21796 · 2021 · External reference

The polycomb group proteins bind throughout the INK4A-ARF locus and are disassociated in senescent cells

10.1101/gad.415507 · 2007 · External reference

Histone H3.3K27M represses

10.1158/1541-7786.mcr-16-0389 · 2017 · External reference

H3K27M mutant glioma: disease definition and biological underpinnings

10.1093/neuonc/noad164 · 2024 · External reference

Tazemetostat for tumors harboring SMARCB1/SMARCA4 or EZH2 alterations: results from NCI-COG pediatric MATCH APEC1621C

10.1093/jnci/djad085 · 2023 · External reference

Unresolved reference

External reference

EZH2 inhibition and myeloid risk: a feature of On-Target biology

2026 · External reference

Unresolved reference

2022 · External reference

Tazemetostat in combination with lenalidomide and rituximab in patients with relapsed/refractory follicular lymphoma: updated phase 1b results of symphony-1 with 22.5 months follow-up

10.1182/blood-2023-179910 · 2023 · External reference

PRMT5 inhibition shows in vitro efficacy against H3K27M-altered diffuse midline glioma, but does not extend survival in vivo

10.1038/s41598-023-48652-x · 2024 · External reference

PRMT5 maintains tumor stem cells to promote pediatric high-grade glioma tumorigenesis

10.1158/1541-7786.mcr-24-0233 · 2025 · External reference

Unresolved reference

2023 · External reference

TIP-09. A phase 0/1 study of PRMT5 inhibitor BMS-986504 in recurrent glioblastoma participants with MTAP deletion

10.1093/neuonc/noaf201.1669 · 2025 · External reference

Epigenetic-targeted treatments for H3K27M-Mutant midline gliomas

10.1007/978-981-15-8104-5_6 · 2021 · External reference

Combined treatment with CBP and BET inhibitors reverses inadvertent activation of detrimental super enhancer programs in DIPG cells

10.1038/s41419-020-02800-7 · 2020 · External reference

Combined inhibition of FACT and BET disrupts transcription to suppress tumor growth in mouse models of diffuse midline glioma

10.1126/scitranslmed.adr1557 · 2026 · External reference

Dual targeting of the epigenome via FACT complex and histone deacetylase is a potent treatment strategy for DIPG

10.1016/j.celrep.2021.108994 · 2021 · External reference

Trotabresib, an oral potent bromodomain and extraterminal inhibitor, in patients with high-grade gliomas: a phase I, "window-of-opportunity" study

10.1093/neuonc/noac263 · 2023 · External reference

Trotabresib (CC-90010) in combination with adjuvant temozolomide or concomitant temozolomide plus radiotherapy in patients with newly diagnosed glioblastoma

2022 · External reference

Imipridones affect tumor bioenergetics and promote cell lineage differentiation in diffuse midline gliomas

10.1093/neuonc/noac041 · 2022 · External reference

ONC201 and imipridones: anti-cancer compounds with clinical efficacy

10.1016/j.neo.2020.09.005 · 2020 · External reference

Dordaviprone: first approval

10.1007/s40265-025-02252-3 · 2026 · External reference

Clinical efficacy of ONC201 in H3K27M-Mutant diffuse midline gliomas is driven by disruption of integrated metabolic and epigenetic pathways

10.1158/2159-8290.cd-23-0131 · 2023 · External reference

Drug delivery strategies for paediatric diffuse midline gliomas

10.1016/j.addr.2025.115695 · 2025 · External reference

Emerging advances in combinatorial treatments of epigenetically altered pediatric high-grade H3K27M gliomas

10.3389/fgene.2021.742561 · 2021 · External reference