H3G34-Mutant Gliomas-A Review of Molecular Pathogenesis and Therapeutic Options

Editorial Material Clinical Neurology

Imaging features of diffuse hemispheric glioma, H3 G34-mutant: Report of 4 cases

Tuba Kalelioglu et al.

JOURNAL OF NEURORADIOLOGY (2023)

Add to Collection

Review Pharmacology & Pharmacy

How to treat histone 3 altered gliomas: molecular landscape and therapeutic developments

Vincenzo Di Nunno et al.

EXPERT REVIEW OF CLINICAL PHARMACOLOGY (2023)

Add to Collection

Article Biochemistry & Molecular Biology

Single substitution in H3.3G34 alters DNMT3A recruitment to cause progressive neurodegeneration

Sima Khazaei et al.

Summary: Germline histone H3.3 amino acid substitutions, including H3.3G34R/V, cause severe neurodevelopmental syndromes. In this study, knock-in mice with H3.3G34R/V/W mutations were generated to investigate their impact on brain development. The H3.3G34R mutation led to progressive microcephaly, neurodegeneration, and abnormal accumulation of disease-associated microglia, along with neuronal depletion.

CELL (2023)

Add to Collection

Article Clinical Neurology

Update on quality assurance in neuropathology: Summary of the round robin trials on TERT promoter mutation, H3-3A mutation, 1p/19q codeletion, and KIAA1549::BRAF fusion testing in Germany in 2020 and 2021

Sandra Pohl et al.

Summary: In 2020 and 2021, neuropathological round robin trials expanded to include IDH mutational testing, MGMT promoter methylation analysis, 1p/19q codeletion testing, and new molecular markers such as TERT promoter mutation, KIAA1549::BRAF fusions, and H3-3A mutations. The success rates in all four trials ranged from 75 to 96%, indicating a high overall quality level in molecular neuropathological diagnostics.

CLINICAL NEUROPATHOLOGY (2023)

Add to Collection

Article Oncology

A Compendium of Syngeneic, Transplantable Pediatric High-Grade Glioma Models Reveals Subtype-Specific Therapeutic Vulnerabilities

Michael McNicholas et al.

Summary: Pediatric high-grade gliomas (pHGG) are heterogeneous brain tumors driven by clonal mutations in histone genes. We developed models representing 16 pHGG subtypes with different genetic alterations, which showed different treatment sensitivities and invasiveness. These models provide evidence for subtype-specific biology and therapeutic targeting in histone-mutant gliomas.

CANCER DISCOVERY (2023)

Add to Collection

Article Oncology

Integrated genetic profiling of archival pediatric high-grade glial tumors and reassessment with 2021 WHO classification of paediatric CNS tumours

Linda D. Cooley et al.

Summary: This study analyzed 33 cases of pediatric high-grade gliomas (pHGG) and found genetic aberrations in all tumors, with 6 cases upgraded from WHO grade 3 to grade 4. New genetic features were discovered, highlighting the potential of small studies to uncover novel genetic characteristics and subtypes.

CANCER GENETICS (2023)

Add to Collection

Article

H3G34-mutant diffuse hemispheric glioma with osseous metastases: a case report and literature review

Nina Yu et al.

CNS oncology (2023)

Add to Collection

Article Oncology

Tumor microenvironment in glioblastoma: Current and emerging concepts

Pratibha Sharma et al.

Summary: Glioblastoma (GBM) tumor microenvironment (TME) is a highly heterogeneous and complex system, which plays a crucial role in the proliferation, invasion, migration, and survival of GBM cells. Interactions between GBM cells and the TME occur through direct cell-to-cell contact and indirect signaling via cytokines, growth factors, and extracellular vehicles. Recent discoveries of neuron-glioma interfaces and neurotransmitter-based interactions have provided insights into novel mechanisms promoting tumor cell growth and survival. This review discusses the communication between GBM cells and the TME, various models for studying the TME, and current therapeutic strategies targeting TME components.

NEURO-ONCOLOGY ADVANCES (2023)

Add to Collection

Article Clinical Neurology

H3 G34-mutant high-grade gliomas: integrated clinical, imaging and pathological characterisation of a single-centre case series

Jose Pedro Lavrador et al.

Summary: This study provides a comprehensive clinical, imaging, and pathological review of diffuse hemispheric glioma with H3 G34R mutation. The results demonstrate that this tumor has characteristic imaging and pathological features, and genomic analysis can offer potential opportunities for personalized treatment and better patient management.

ACTA NEUROCHIRURGICA (2023)

Add to Collection

Article Oncology

Novel genetically engineered H3.3G34R model reveals cooperation with ATRX loss in upregulation of Hoxa cluster genes and promotion of neuronal lineage

Aalaa S. Abdallah et al.

Summary: This study developed an animal model of pediatric high-grade gliomas (pHGGs) to investigate the downstream effects of the H3.3G34R mutation. The results showed that ATRX loss significantly increased tumor latency and inhibited ependymal differentiation in the presence of H3.3G34R. Transcriptomic analysis revealed that ATRX loss upregulated Hoxa cluster genes in H3.3G34R pHGGs and the overexpression of H3.3G34R enriched neuronal markers only in the context of ATRX loss.

NEURO-ONCOLOGY ADVANCES (2023)

Add to Collection

Review Oncology

The prognostic significance of further genotyping H3G34 diffuse hemispheric gliomas

Huy Gia Vuong et al.

Summary: This study investigated the frequency of genetic events in H3G34-mutant DHG and their association with survival trends. The researchers found that H3G34V mutation had a worse prognosis than H3G34R mutation, TP53 mutation was the most common genetic alteration, and PDGFRA amplification or EGFR amplification led to poor survival.

CANCER (2022)

Add to Collection

Review Clinical Neurology

Neuroimaging features of diffuse hemispheric glioma, H3 G34-mutant: A case series and systematic review

Ryo Kurokawa et al.

Summary: Diffuse hemispheric gliomas, H3 G34-mutant (DHGs-G34m), predominantly occur in the supratentorial regions of adolescents, with prognosis varying among patients. Evaluation of tumor margins may have prognostic value.

JOURNAL OF NEUROIMAGING (2022)

Add to Collection

Article Medicine, Research & Experimental

High frequency of PDGFRA and MUC family gene mutations in diffuse hemispheric glioma, H3 G34-mutant: a glimmer of hope?

Wanming Hu et al.

Summary: This study reports the clinicopathological, molecular, and immune infiltration characteristics of Chinese patients with G34-DHG and compares them with cases from the HERBY Trial and CGGA database. The results demonstrate a high frequency of PDGFRA and MUC gene family mutations in G34-DHG, with PDGFRA mutation serving as an indicator of poor prognosis and MUC16 mutation indicating favorable prognosis and high immune infiltration. These findings provide new directions for targeted therapy and immunotherapy in G34-DHG patients.

JOURNAL OF TRANSLATIONAL MEDICINE (2022)

Add to Collection

Article Oncology

Germline MUTYH mutations and high-grade gliomas: Novel evidence for a potential association

Gabor Bedics et al.

Summary: There is growing evidence supporting the role of germline mutations in pediatric central nervous system tumors, and next-generation sequencing panels can help detect them. MUTYH gene variants are now known to be associated with various extraintestinal cancers, in addition to their role in polyposis syndrome. Using a multigene next-generation sequencing panel, two mutant pediatric high-grade diffuse gliomas were identified along with germline MUTYH variants. These findings suggest a possible association between germline MUTYH mutations and CNS malignancies, particularly in pediatric histone H3-mutant gliomas.

GENES CHROMOSOMES & CANCER (2022)

Add to Collection

Article Multidisciplinary Sciences

Molecular alterations in the integrated diagnosis of pediatric glial and glioneuronal tumors: A single center experience

Sandra Lorena Colli et al.

Summary: This study evaluated molecular alterations in BRAF and histone 3 genes in pediatric glial and glioneuronal tumors and correlated the results with clinical and histological findings. The study found that certain gene mutations were associated with specific tumor types and survival rates, providing insights for more accurate diagnosis and treatment.

PLOS ONE (2022)

Add to Collection

Article Genetics & Heredity

A Saccharomyces cerevisiae model and screen to define the functional consequences of oncogenic histone missense mutations

Laramie D. Lemon et al.

Summary: Missense mutations in histone genes can transform essential proteins into oncogenes, driving oncogenesis. By studying the budding yeast model, researchers have identified the functional consequences of several oncohistone mutations, as well as genes associated with histone function and transcriptional regulation.

G3-GENES GENOMES GENETICS (2022)

Add to Collection

Review Biochemistry & Molecular Biology

The Intricate Epigenetic and Transcriptional Alterations in Pediatric High-Grade Gliomas: Targeting the Crosstalk as the Oncogenic Achilles' Heel

Paul Huchede et al.

Summary: This article reviews the molecular specificities of pediatric high-grade gliomas (pHGGs) in the context of epigenomic rewiring and the co-opted transcriptional signaling pathways. Understanding the synergistic effects of transcriptional and epigenetic alterations in these tumors can help identify new therapeutic targets.

BIOMEDICINES (2022)

Add to Collection

Article Clinical Neurology

CTNI-31. COG ACNS1721: PHASE 2 STUDY OF VELIPARIB AND LOCAL IRRADIATION, FOLLOWED BY MAINTENANCE VELIPARIB AND TEMOZOLOMIDE, IN PATIENTS WITH NEWLY DIAGNOSED HIGH-GRADE GLIOMA WITHOUT H3 K27M OR BRAF MUTATIONS

Matthias Karajannis et al.

NEURO-ONCOLOGY (2022)

Add to Collection

Article Clinical Neurology

Oncohistone interactome profiling uncovers contrasting oncogenic mechanisms and identifies potential therapeutic targets in high grade glioma

Robert Siddaway et al.

Summary: Histone H3 mutations at amino acids 27 (H3K27M) and 34 (H3G34R) drive pediatric-type high-grade glioma (pHGG) by disrupting chromatin modifications and altering cellular processes. H3K9 methylation plays a crucial role in regulating the effects of these mutations and could be a potential therapeutic target.

ACTA NEUROPATHOLOGICA (2022)

Add to Collection

Article Oncology

Pediatric glioblastoma cells are sensitive to drugs that inhibit eIF2α dephosphorylation and its phosphomimetic S51D variant

Karin Eytan et al.

Summary: This study found that pediatric glioblastoma (PED-GBM) cells are sensitive to the combination of histone deacetylase inhibitors and PARP-1 inhibitors. Increased phosphorylation of eIF2 alpha (P-eIF2 alpha) plays a role in modulating PED-GBM survival and response to treatments, and certain drugs can enhance the effect of increased P-eIF2 alpha. These drugs also increase the sensitivity of PED-GBM cells to radiation. Furthermore, PED-GBM is more sensitive to these drugs compared to normal human astrocytes.

FRONTIERS IN ONCOLOGY (2022)

Add to Collection

Review Medicine, General & Internal

Extent of Tumor Resection and Survival in Pediatric Patients With High-Grade Gliomas A Systematic Review and Meta-analysis

Rami Hatoum et al.

Summary: The study found that in pediatric patients with high-grade gliomas, gross total resection (GTR) is independently associated with better overall survival compared with subtotal resection (STR) and biopsy, especially among patients with hemispheric and infratentorial tumors, supporting the pursuit of maximal safe resection in the treatment of pHGGs.

JAMA NETWORK OPEN (2022)

Add to Collection

Article

Utility of arterial spin labeling for objective assessment of intratumoral microvessels in diffuse hemispheric glioma, H3 G34R-mutant: A case report and literature review

Kei Kitakami et al.

Radiology Case Reports (2022)

Add to Collection

Review Pathology

Paediatric-type diffuse high-grade gliomas in the 5th CNS WHO Classification

Francesca Gianno et al.

Summary: This article focuses on the unique pathobiology of paediatric-type diffuse high-grade gliomas, which are recognized for the first time in the fifth CNS WHO Classification. It discusses the diagnostic criteria and differential diagnosis for each tumour type within this family, with a specific emphasis on the challenges encountered in daily practice, particularly in diagnosing diffuse paediatric-type high-grade glioma H3-wildtype and IDH-wildtype. The advantages and limitations of molecular tests for defining entities within this tumour family are evaluated in different diagnostic contexts.

PATHOLOGICA (2022)

Add to Collection

Article Medicine, Research & Experimental

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

Santiago Haase et al.

Summary: This study explores the mechanisms of the H3.3-G34R/V gene mutation in pediatric high-grade gliomas (pHGGs) and its effects on DNA repair pathways and immune response. Combination therapy involving radiotherapy, DNA damage response inhibitors, and STING agonists was found to be effective in treating pHGGs with G34 mutations, leading to long-term survival and immunological memory.

JOURNAL OF CLINICAL INVESTIGATION (2022)

Add to Collection

Article Oncology

The dominant TP53 hotspot mutation in IDH-mutant astrocytoma, R273C, has distinctive pathologic features and sex-specific prognostic implications

Daniel F. Marker et al.

Summary: Our study found that in IDH-mutant astrocytomas, the TP53 mutational spectrum is predominantly driven by a single hotspot mutation, which is not commonly found in IDH-wildtype astrocytomas. Tumors with TP53(R273C) mutation exhibit lower proliferation but tend to progress more quickly and have shorter survival, especially in male patients.

NEURO-ONCOLOGY ADVANCES (2022)

Add to Collection

Article Oncology

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

Cameron Crowell et al.

Summary: This systematic review provides a comprehensive overview of clinical data for G34-DHG, highlighting poor prognosis and important prognostic features. It serves as a baseline for future research and clinical trials.

NEURO-ONCOLOGY ADVANCES (2022)

Add to Collection

Article Clinical Neurology

EvaluatingH3F3AK27M and G34R/V somatic mutations in a cohort of pediatric brain tumors of different and rare histologies

Vinicius Fernandes Oliveira et al.

Summary: This study investigated somatic mutations on H3 histone in pediatric brain tumors, finding a higher frequency of H3F3A/K27M mutations in HGGs compared to other tumor types. No H3F3A/G34R/V mutations were observed in the samples.

CHILDS NERVOUS SYSTEM (2021)

Add to Collection

Article Clinical Neurology

Morphological diversity in H3G34-mutant high-grade gliomas: Ganglionic and epithelioid features

Ahmed Gilani et al.

Summary: Studies on H3G34 mutant tumors have revealed morphological diversity, including advanced ganglionic differentiation and epithelioid features.

CLINICAL NEUROPATHOLOGY (2021)

Add to Collection

Article Oncology

H3 G34-mutant high-grade glioma

Ka Young Lim et al.

Summary: H3.3G34-mutant high-grade gliomas (HGG) exhibit uniform genetic and epigenetic characteristics, with a median survival rate of 23.5 months. The study suggests that tumor-infiltrating area and resectability may be crucial parameters for the prognosis of patients.

BRAIN TUMOR PATHOLOGY (2021)

Add to Collection

Article Pathology

Correlation Between Immunohistochemistry and Sequencing in H3G34-Mutant Gliomas

Francesca Gianno et al.

Summary: Recurrent glycine-to-arginine/valine alterations at codon 34 (G34R/V) within H3F3A gene characterize a subset of hemispheric high-grade gliomas (HGG) affecting children and young adults. The value of immunohistochemistry (IHC) to detect G34R/V-mutated cases is not highly predictive and confirmation by sequencing is mandatory; G34R/V mutations should be suspected in all hemispheric tumor IDH1/2 wild type, showing loss of OLIG2 and ATRX and/or p53 expression.

AMERICAN JOURNAL OF SURGICAL PATHOLOGY (2021)

Add to Collection

Article Clinical Neurology

C19MC amplification and expression of Lin28A and Olig2 in the classification of embryonal tumors of the central nervous system: A 14-year retrospective study from a tertiary care center

Aruna Nambirajan et al.

Summary: CET is a group of tumors with poorly defined diagnostic criteria, diagnosed mainly by exclusion of other molecularly defined entities and histological mimics. A subset of CET can be subclassified using markers such as C19MC amplification, Lin28A, and Olig2, but the diagnostic specificity of these markers is limited and should be used in conjunction with other diagnostic methods.

CHILDS NERVOUS SYSTEM (2021)

Add to Collection

Letter Clinical Neurology

Post-treatment hypermutation in a recurrent diffuse glioma with H3.3 p.G34 Mutation

Matthew D. Wood et al.

NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY (2021)

Add to Collection

Article Cell & Tissue Engineering

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

Raul Bardini Bressan et al.

Summary: Point mutations within histone H3.3 are frequent in pediatric high-grade gliomas (pHGGs), with distinct mutations arising in different brain regions such as the forebrain and hindbrain. Regional differences in the response of fetal neural stem cells to these mutations suggest a possible role in tumorigenesis and tumor development.

CELL STEM CELL (2021)

Add to Collection

Article Cell & Tissue Engineering

Dissecting the impact of regional identity and the oncogenic role of human-specific NOTCH2NL in an hESC model of H3.3G34R-mutant glioma

Kosuke Funato et al.

Summary: The study reveals that the H3.3G34R-mutant glioma model recapitulates key features of the tumors, showing specificity to forebrain interneuronal progenitors; Mutations in H3.3G34R, ATRX, and TP53 cooperatively impact RNA splicing events and lead to increased expression of components of the Notch pathway, particularly NOTCH2NL gene family.

CELL STEM CELL (2021)

Add to Collection

Article Cell Biology

Epigenetically defined therapeutic targeting in H3.3G34R/V high-grade gliomas

Stefan R. Sweha et al.

Summary: High-grade gliomas with H3.3G34R/V mutations have poor prognosis and current treatments are not curative. Epigenetic analysis revealed the LIF/STAT3 pathway as a key vulnerability in H3.3G34R/V gliomas, suggesting potential for targeted therapies.

SCIENCE TRANSLATIONAL MEDICINE (2021)

Add to Collection

Article Biology

Suprising phenotypic diversity of cancer-associated mutations of Gly 34 in the histone H3 tail

Brandon R. Lowe et al.

Summary: The study highlights the varied phenotypic effects and impact on DNA repair caused by mutations in different types of H3 histones, indicating the complexity associated with substitutions at a single residue in H3. Analysis using genetically tractable systems demonstrates the utility of such approaches in studying these mutations.

ELIFE (2021)

Add to Collection

Article Neurosciences

Molecular analysis of pediatric CNS-PNET revealed nosologic heterogeneity and potent diagnostic markers for CNS neuroblastoma with FOXR2-activation

Andrey Korshunov et al.

Summary: This study identified molecular markers, namely SOX10 and ANKRD55, as key genes distinguishing CNS_NBL from other tumors exhibiting CNS-PNET. Immunohistochemical detection of combined expression of SOX10 and ANKRD55 accurately identifies CNS_NBL, a distinct pediatric brain tumor entity, from morphological mimics. Prospective patient series are needed to confirm the diagnostic IHC algorithm due to the rarity of CNS_NBL.

ACTA NEUROPATHOLOGICA COMMUNICATIONS (2021)

Add to Collection

Review Oncology

H3K36 trimethylation-mediated biological functions in cancer

Chu Xiao et al.

Summary: Histone H3 lysine 36 trimethylation (H3K36me3) plays a crucial role in genome regulation, especially in cancer pathogenesis. Disruption of the histone methylation regulatory network and mutations in H3K36me3 codes may have a powerful impact on cancer initiation and development. Understanding the role of H3K36me3 in cancer can lead to targeted therapeutic strategies for cancer treatment.

CLINICAL EPIGENETICS (2021)

Add to Collection

Review Clinical Neurology

Advanced Magnetic Resonance Imaging in Pediatric Glioblastomas

Fabricio Guimaraes Goncalves et al.

Summary: The soon-to-be-released 5th edition of the World Health Organization Classification of Tumors of the Central Nervous System is making extensive changes in the terminology regarding diffuse high-grade gliomas. Glioblastomas, as an entity, are now classified differently, with separate categories for pediatric and adult types. The standard care for pediatric glioblastomas is still uncertain, with surgery usually being the initial treatment option.

FRONTIERS IN NEUROLOGY (2021)

Add to Collection

Article Multidisciplinary Sciences

Mutations inhibiting KDM4B drive ALT activation in ATRX-mutated glioblastomas

M. Udugama et al.

Summary: The study reveals the functional role of histone demethylases KDM4B in regulating ALT activation, and identifies H3.3(G34R) and IDH1/2 mutations as cooperating factors with ATRX mutations to promote ALT in glioblastomas.

NATURE COMMUNICATIONS (2021)

Add to Collection

Review Cell Biology

Mechanisms of Cellular Senescence: Cell Cycle Arrest and Senescence Associated Secretory Phenotype

Ruchi Kumari et al.

Summary: Cellular senescence is a stable cell cycle arrest triggered in response to various stimuli, characterized by dynamic changes in senescent cells. It has both positive effects on tissue repair and anti-cancer mechanisms, but can also negatively impact organismal health. Multiple pathways, including p53/p21 and p16/pRB, play central roles in regulating this process.

FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY (2021)

Add to Collection

Article Clinical Neurology

Malignant gliomas with H3F3A G34R mutation or MYCN amplification in pediatric patients with Li Fraumeni syndrome

Melanie Schoof et al.

ACTA NEUROPATHOLOGICA (2021)

Add to Collection

Article Pathology

Histone H3.3 G34-mutant Diffuse Gliomas in Adults

Leiming Wang et al.

AMERICAN JOURNAL OF SURGICAL PATHOLOGY (2021)

Add to Collection

Review Oncology

The 2021 WHO Classification of Tumors of the Central Nervous System: a summary

David N. Louis et al.

Summary: The fifth edition of the WHO Classification of Tumors of the Central Nervous System integrates molecular diagnostics into CNS tumor classification, introduces different approaches to tumor nomenclature and grading, and emphasizes the importance of integrated diagnoses and layered reports.

NEURO-ONCOLOGY (2021)

Add to Collection

Article Clinical Neurology

KS01.3.A Tumoral MHC class II expression in gliomas drives T cell exhaustion

Y Chih et al.

NEURO-ONCOLOGY (2021)

Add to Collection

Letter Oncology

Diffuse hemispheric glioma, H3 G34-mutant: Genomic landscape of a new tumor entity and prospects for targeted therapy

Calixto-Hope G. Lucas et al.

NEURO-ONCOLOGY (2021)

Add to Collection

Article Clinical Neurology

Alternative lengthening of telomeres in molecular subgroups of paediatric high-grade glioma

Simone Minasi et al.

Summary: The study found a strong association between H3.3 mutations and ALT mechanism, highlighting different telomere profiles among histone-defined subgroups, suggesting that acquiring the Gly34 mutation on H3.3 might be sufficient to trigger the ALT mechanism.

CHILDS NERVOUS SYSTEM (2021)

Add to Collection

Article Oncology

Radiological Evaluation of Newly Diagnosed Non-Brainstem Pediatric High-Grade Glioma in the HERBY Phase II Trial

Daniel Rodriguez Gutierrez et al.

CLINICAL CANCER RESEARCH (2020)

Add to Collection

Editorial Material Radiology, Nuclear Medicine & Medical Imaging

Extracranial Metastases From Glioblastoma With Primitive Neuronal Components on FDG PET/CT

Matthew D. Kay et al.

CLINICAL NUCLEAR MEDICINE (2020)

Add to Collection

Article Oncology

High-grade gliomas in adolescents and young adults highlight histomolecular differences from their adult and pediatric counterparts

Alexandre Roux et al.

NEURO-ONCOLOGY (2020)

Add to Collection

Article Multidisciplinary Sciences

RACK7 recognizes H3.3G34R mutation to suppress expression of MHC class II complex components and their delivery pathway in pediatric glioblastoma

Fangfang Jiao et al.

SCIENCE ADVANCES (2020)

Add to Collection

Article Oncology

Children with DIPG and high-grade glioma treated with temozolomide, irinotecan, and bevacizumab: the Seattle Children's Hospital experience

Erin E. Crotty et al.

JOURNAL OF NEURO-ONCOLOGY (2020)

Add to Collection

Article Clinical Neurology

Low-Grade Gemistocytic Morphology in H3 G34R-Mutant Gliomas and Concurrent K27M Mutation: Clinicopathologic Findings

Meaghan Morris et al.

JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2020)

Add to Collection

Article Multidisciplinary Sciences

Histone H3.3 G34 mutations promote aberrant PRC2 activity and drive tumor progression

Siddhant U. Jain et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2020)

Add to Collection

Article Biochemistry & Molecular Biology

Histone H3.3G34-Mutant Intemeuron Progenitors Co-opt PDGFRA for Gliomagenesis

Carol C. L. Chen et al.

CELL (2020)

Add to Collection

Article Multidisciplinary Sciences

PRC2 and EHMT1 regulate H3K27me2 and H3K27me3 establishment across the zygote genome

Tie-Gang Meng et al.

NATURE COMMUNICATIONS (2020)

Add to Collection

Article Neurosciences

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

Tina Yi-Ting Huang et al.

ACTA NEUROPATHOLOGICA COMMUNICATIONS (2020)

Add to Collection

Review Clinical Neurology

Beyond the World Health Organization classification of central nervous system tumors 2016: what are the new developments for gliomas from a clinician's perspective?

Michael Weller et al.

CURRENT OPINION IN NEUROLOGY (2020)

Add to Collection

Article Multidisciplinary Sciences

Globally altered epigenetic landscape and delayed osteogenic differentiation in H3.3-G34W-mutant giant cell tumor of bone

Pavlo Lutsik et al.

NATURE COMMUNICATIONS (2020)

Add to Collection

Review Clinical Neurology

Radiological and Immunostaining Characteristics of H3.3 G34R-Mutant Glioma: A Report of 3 Cases and Review of the Literature

Shumpei Onishi et al.

PEDIATRIC NEUROSURGERY (2020)

Add to Collection

Article Pathology

Cerebral hemispheric glioblastoma with PNET-like morphology and histone H3.3 G34 mutation in younger patients: Report of three rare cases and diagnostic pitfalls

Yuanyuan Cheng et al.

INDIAN JOURNAL OF PATHOLOGY AND MICROBIOLOGY (2020)

Add to Collection

Review Pathology

Applications of molecular neuro-oncology - a review of diffuse glioma integrated diagnosis and emerging molecular entities

Matthew D. Wood et al.

DIAGNOSTIC PATHOLOGY (2019)

Add to Collection

Article Neurosciences

H3F3A-G34R mutant high grade neuroepithelial neoplasms with glial and dysplastic ganglion cell components

Felipe Andreiuolo et al.

ACTA NEUROPATHOLOGICA COMMUNICATIONS (2019)

Add to Collection

Review Oncology

Histone H3 Mutations: An Updated View of Their Role in Chromatin Deregulation and Cancer

Brandon R. Lowe et al.

CANCERS (2019)

Add to Collection

Article Oncology

Extraneural Metastases From a High-Grade Glioma (HGG) With an H3F3A G34R Mutation

Amit Jethanandani et al.

FRONTIERS IN ONCOLOGY (2019)

Add to Collection

Review Oncology

Anaplastic pleomorphic xanthoastrocytoma associated with an H3G34 mutation: a case report with review of literature

Shoh Sasaki et al.

BRAIN TUMOR PATHOLOGY (2019)

Add to Collection

Article Oncology

CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2012-2016

Quinn T. Ostrom et al.

NEURO-ONCOLOGY (2019)

Add to Collection

Article Oncology

Direct Targeting of MYCN Gene Amplification by Site-Specific DNA Alkylation in Neuroblastoma

Hiroyuki Yoda et al.

CANCER RESEARCH (2019)

Add to Collection

Review Biochemistry & Molecular Biology

Targeting epigenetic regulators for cancer therapy: mechanisms and advances in clinical trials

Yuan Cheng et al.

SIGNAL TRANSDUCTION AND TARGETED THERAPY (2019)

Add to Collection

Article Clinical Neurology

Low FoxG1 and high Olig-2 labelling indices define a prognostically favourable subset in isocitrate dehydrogenase (IDH)-mutant gliomas

S. Schaefer et al.

NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY (2018)

Add to Collection

Article Oncology

Molecular, Pathological, Radiological, and Immune Profiling of Non-brainstem Pediatric High-Grade Glioma from the HERBY Phase II Randomized Trial

Alan Mackay et al.

CANCER CELL (2018)

Add to Collection

Article Biochemistry & Molecular Biology

Rpp29 regulates histone H3.3 chromatin assembly through transcriptional mechanisms

Prashanth Krishna Shastrula et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2018)

Add to Collection

Article Oncology

Phase II, Open-Label, Randomized, Multicenter Trial (HERBY) of Bevacizumab in Pediatric Patients With Newly Diagnosed High-Grade Glioma

Jacques Grill et al.

JOURNAL OF CLINICAL ONCOLOGY (2018)

Add to Collection

Article Clinical Neurology

Historadiological correlations in high-grade glioma with the histone 3.3 G34R mutation

Julien Puntonet et al.

JOURNAL OF NEURORADIOLOGY (2018)

Add to Collection

Review Oncology

Molecular classification of adult gliomas: recent advances and future perspectives

Marc Barritault et al.

CURRENT OPINION IN ONCOLOGY (2018)

Add to Collection

Article Multidisciplinary Sciences

Cancer-driving H3G34V/R/D mutations block H3K36 methylation and H3K36me3-MutS alpha interaction

Jun Fang et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2018)

Add to Collection

Article Multidisciplinary Sciences

Inhibition of a K9/K36 demethylase by an H3.3 point mutation found in paediatric glioblastoma

Hsiao P. J. Voon et al.

NATURE COMMUNICATIONS (2018)

Add to Collection

Article Radiology, Nuclear Medicine & Medical Imaging

Characterization of Diffuse Gliomas With Histone H3-G34 Mutation by MRI and Dynamic 18F-FET PET

Franziska J. Vettermann et al.

CLINICAL NUCLEAR MEDICINE (2018)

Add to Collection

Article Oncology

Extensive Molecular and Clinical Heterogeneity in Patients With Histologically Diagnosed CNS-PNET Treated as a Single Entity: A Report From the Children's Oncology Group Randomized ACNS0332 Trial

Eugene I. Hwang et al.

JOURNAL OF CLINICAL ONCOLOGY (2018)

Add to Collection

Article Multidisciplinary Sciences

Transcriptome and protein interaction profiling in cancer cells with mutations in histone H3.3

Jinyeong Lim et al.

SCIENTIFIC DATA (2018)

Add to Collection

Review Allergy

Histone modifications and their role in epigenetics of atopy and allergic diseases

Bilal Alaskhar Alhamwe et al.

ALLERGY ASTHMA AND CLINICAL IMMUNOLOGY (2018)

Add to Collection

Article Neurosciences

Molecular Diagnostic and Prognostic Subtyping of Gliomas in Tunisian Population

Saoussen Trabelsi et al.

MOLECULAR NEUROBIOLOGY (2017)

Add to Collection

Article Multidisciplinary Sciences

Molecular basis for the role of oncogenic histone mutations in modulating H3K36 methylation

Yinglu Zhang et al.

SCIENTIFIC REPORTS (2017)

Add to Collection

Article Biology

Histone H3G34R mutation causes replication stress, homologous recombination defects and genomic instability in S. pombe

Rajesh K. Yadav et al.

ELIFE (2017)

Add to Collection

Article Neurosciences

Evaluation of a novel antibody to define histone 3.3 G34R mutant brain tumours

Farhana Haque et al.

ACTA NEUROPATHOLOGICA COMMUNICATIONS (2017)

Add to Collection

Article Neurosciences

Detection of Histone H3 mutations in cerebrospinal fluid-derived tumor DNA from children with diffuse midline glioma

Tina Y. Huang et al.

ACTA NEUROPATHOLOGICA COMMUNICATIONS (2017)

Add to Collection

Article Neurosciences

Characterizing temporal genomic heterogeneity in pediatric high-grade gliomas

Ralph Salloum et al.

ACTA NEUROPATHOLOGICA COMMUNICATIONS (2017)

Add to Collection

Article Oncology

Integrated Molecular Meta-Analysis of 1,000 Pediatric High-Grade and Diffuse Intrinsic Pontine Glioma

Alan Mackay et al.

CANCER CELL (2017)

Add to Collection

Article Oncology

Prevalence and clinicopathological features of H3.3 G34-mutant high-grade gliomas: a retrospective study of 411 consecutive glioma cases in a single institution

Koji Yoshimoto et al.

BRAIN TUMOR PATHOLOGY (2017)

Add to Collection

Article Clinical Neurology

Histologically distinct neuroepithelial tumors with histone 3 G34 mutation are molecularly similar and comprise a single nosologic entity

Andrey Korshunov et al.

ACTA NEUROPATHOLOGICA (2016)

Add to Collection

Article Clinical Neurology

Gliomatosis cerebri: no evidence for a separate brain tumor entity

Ulrich Herrlinger et al.

ACTA NEUROPATHOLOGICA (2016)

Add to Collection

Article Oncology

A pilot study of bevacizumab-based therapy in patients with newly diagnosed high-grade gliomas and diffuse intrinsic pontine gliomas

Trent R. Hummel et al.

JOURNAL OF NEURO-ONCOLOGY (2016)

Add to Collection

Article Oncology

Biomarker-based prognostic stratification of young adult glioblastoma

Rui-qi Zhang et al.

ONCOTARGET (2016)

Add to Collection

Article Clinical Neurology

Gliomatosis cerebri in children shares molecular characteristics with other pediatric gliomas

Alberto Broniscer et al.

ACTA NEUROPATHOLOGICA (2016)

Add to Collection

Article Cell Biology

Molecular basis for oncohistone 113 recognition by SETD2 methyltransferase

Shuang Yang et al.

GENES & DEVELOPMENT (2016)

Add to Collection

Article Clinical Neurology

Distinct Histomorphology in Molecular Subgroups of Glioblastomas in Young Patients

Julia E. Neumann et al.

JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY (2016)

Add to Collection

Article Biochemistry & Molecular Biology

Recurrent MET fusion genes represent a drug target in pediatric glioblastoma

Sebastian Bender et al.

NATURE MEDICINE (2016)

Add to Collection

Article Neurosciences

ATRX immunostaining predicts IDH and H3F3A status in gliomas

Azadeh Ebrahimi et al.

ACTA NEUROPATHOLOGICA COMMUNICATIONS (2016)

Add to Collection

Article Plant Sciences

H3K36ac Is an Evolutionary Conserved Plant Histone Modification That Marks Active Genes

Walid Mahrez et al.

PLANT PHYSIOLOGY (2016)

Add to Collection

Article Clinical Neurology

Integrated analysis of pediatric glioblastoma reveals a subset of biologically favorable tumors with associated molecular prognostic markers

Andrey Korshunov et al.

ACTA NEUROPATHOLOGICA (2015)

Add to Collection

Article Clinical Neurology

Adult IDH wild type astrocytomas biologically and clinically resolve into other tumor entities

David E. Reuss et al.

ACTA NEUROPATHOLOGICA (2015)

Add to Collection

Article Oncology

Altered global histone-trimethylation code and H3F3A-ATRX mutation in pediatric GBM

Pankaj Pathak et al.

JOURNAL OF NEURO-ONCOLOGY (2015)

Add to Collection

Article Oncology

Molecular analysis of diffuse intrinsic brainstem gliomas in adults

German Reyes-Botero et al.

JOURNAL OF NEURO-ONCOLOGY (2014)

Add to Collection

Article Genetics & Heredity

The genomic landscape of diffuse intrinsic pontine glioma and pediatric non-brainstem high-grade glioma

Gang Wu et al.

NATURE GENETICS (2014)

Add to Collection

Article Genetics & Heredity

Recurrent somatic mutations in ACVR1 in pediatric midline high-grade astrocytoma

Adam M. Fontebasso et al.

NATURE GENETICS (2014)

Add to Collection

Article Neurosciences

The prognostic role of intragenic copy number breakpoints and identification of novel fusion genes in paediatric high grade glioma

Diana Carvalho et al.

ACTA NEUROPATHOLOGICA COMMUNICATIONS (2014)

Add to Collection

Article Clinical Neurology

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

Adam M. Fontebasso et al.

ACTA NEUROPATHOLOGICA (2013)

Add to Collection

Article Oncology

Novel Oncogenic PDGFRA Mutations in Pediatric High-Grade Gliomas

Barbara S. Paugh et al.

CANCER RESEARCH (2013)

Add to Collection

Article Cell Biology

A lesson learned from the H3.3K27M mutation found in pediatric glioma A new approach to the study of the function of histone modifications in vivo?

Kui Ming Chan et al.

CELL CYCLE (2013)

Add to Collection

Article Oncology

H3.3 G34R mutations in pediatric primitive neuroectodermal tumors of central nervous system (CNS-PNET) and pediatric glioblastomas: possible diagnostic and therapeutic implications?

Marco Gessi et al.

JOURNAL OF NEURO-ONCOLOGY (2013)

Add to Collection

Article Multidisciplinary Sciences

Inhibition of PRC2 Activity by a Gain-of-Function H3 Mutation Found in Pediatric Glioblastoma

Peter W. Lewis et al.

SCIENCE (2013)

Add to Collection

Article Oncology

Histone H3.3 Mutations Drive Pediatric Glioblastoma through Upregulation of MYCN

Lynn Bjerke et al.

CANCER DISCOVERY (2013)

Add to Collection

Article Oncology

Hotspot Mutations in H3F3A and IDH1 Define Distinct Epigenetic and Biological Subgroups of Glioblastoma

Dominik Sturm et al.

CANCER CELL (2012)

Add to Collection

Article Genetics & Heredity

Epigenetic modifications in cancer

R. Kanwal et al.

CLINICAL GENETICS (2012)

Add to Collection

Article Multidisciplinary Sciences

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

Jeremy Schwartzentruber et al.

NATURE (2012)

Add to Collection

Article Genetics & Heredity

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

Gang Wu et al.

NATURE GENETICS (2012)

Add to Collection

Article Multidisciplinary Sciences

Mesenchymal Transition and PDGFRA Amplification/Mutation Are Key Distinct Oncogenic Events in Pediatric Diffuse Intrinsic Pontine Gliomas

Stephanie Puget et al.

PLOS ONE (2012)

Add to Collection

Article Biochemical Research Methods

Structures of human nucleosomes containing major histone H3 variants

Hiroaki Tachiwana et al.

ACTA CRYSTALLOGRAPHICA SECTION D-BIOLOGICAL CRYSTALLOGRAPHY (2011)

Add to Collection

Review Cell Biology

Regulation of chromatin by histone modifications

Andrew J. Bannister et al.

CELL RESEARCH (2011)

Add to Collection

Review Genetics & Heredity

The Role of the Leukemia Inhibitory Factor (LIF) - Pathway in Derivation and Maintenance of Murine Pluripotent Stem Cells

Urs Graf et al.

GENES (2011)

Add to Collection

Article Cell Biology

TP53 Mutations in Human Cancers: Origins, Consequences, and Clinical Use

Magali Olivier et al.

COLD SPRING HARBOR PERSPECTIVES IN BIOLOGY (2010)

Add to Collection

Article Medicine, General & Internal

Recurrent rearrangements of chromosome 1q21.1 and variable pediatric phenotypes

Heather C. Mefford et al.

NEW ENGLAND JOURNAL OF MEDICINE (2008)

Add to Collection

Article Biochemistry & Molecular Biology

Identification of histone H3 lysine 36 acetylation as a highly conserved histone modification

Stephanie A. Morris et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2007)

Add to Collection

Article Medicine, General & Internal

Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma

R Stupp et al.

NEW ENGLAND JOURNAL OF MEDICINE (2005)

Add to Collection

Article Medicine, Research & Experimental

Downregulation of major histocompatibility complex antigens in invading glioma cells: stealth invasion of the brain

D Zagzag et al.

LABORATORY INVESTIGATION (2005)

Add to Collection

Article Neurosciences

Regulation of MHC class II expression in glioma cells by class II transactivator (CIITA)

Y Takamura et al.

GLIA (2004)

Add to Collection

Article Neurosciences

The caudal ganglionic eminence is a source of distinct cortical and subcortical cell populations

S Nery et al.

NATURE NEUROSCIENCE (2002)

Add to Collection

H3G34-Mutant Gliomas-A Review of Molecular Pathogenesis and Therapeutic Options

Related references

Export Citation

Share Paper