Epigenetic reprogramming and chromatin accessibility in pediatric diffuse intrinsic pontine gliomas: a neural developmental disease
Year: 2019
Type: review
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.
Source: Neuro-Oncology
Language: English
Subfield: Genetics
Field: Medicine
Domain: Health Sciences
Open Access status: bronze
Awards: G1686049839, Novel combined immunotherapeutic strategies for glioma: using pet dogs as a large animal spontaneous model, Immune-mediated therapies in a genetically engineered murine model of diffuse intrinsic pontine glioma, G4560859322, G5197722314, Mechanisms of glioma growth and invasion novel therapeutic strategies, G5561615526, G5787706606, G5932715242, G645032831, Molecular mechanisms mediated by ACVR1 G328V and H3K27M in diffuse intrinsic pontine glioma biology, Functional Dissection of the K27M Histone Mutation In Gliomagenesis, G7219587584, Tuning Biomaterials-immune cell interactions for treatment of glioblastoma multiforme, G842143505, Interactions between the tumor cells and the neuro-immune microenvironment in mutant IDH1 gliomas: implications for therapeutics, G9000056385, G9000085971, G9000170857, University of Michigan Rogel Cancer Center Support Grant 2023-2028, Oligodendrocyte-focused rAAV gene therapy strategies for Canavan disease and Leukodystrophies
OpenAlex ID: W2989092138