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EZH2 Inhibition Sensitizes IDH1R132H-Mutant Gliomas to Histone Deacetylase Inhibitor

Title: EZH2 Inhibition Sensitizes IDH1R132H-Mutant Gliomas to Histone Deacetylase Inhibitor
Authors: Sprinzen, Lisa; Garcia, Franklin; Mela, Angeliki; Lei, Liang; Upadhyayula, Pavan; Mahajan, Aayushi; Humala, Nelson; Manier, Lisa; Caprioli, Richard; Quiñones-Hinojosa, Alfredo; Casaccia, Patrizia; Canoll, Peter
Source: Cells, vol 13, iss 3
Publisher Information: eScholarship, University of California
Publication Year: 2024
Collection: University of California: eScholarship
Subject Terms: Biomedical and Clinical Sciences; Oncology and Carcinogenesis; Brain Cancer; Neurosciences; Brain Disorders; Orphan Drug; Cancer; Rare Diseases; Genetics; 2.1 Biological and endogenous factors; 6.1 Pharmaceuticals; Animals; Mice; Histone Deacetylase Inhibitors; Histones; Glioma; Benzamides; Biphenyl Compounds; Morpholines; Pyridones; EZH2; H3K27; HDAC; IDH1; OPC; gliomagenesis; Biological sciences
Description: Isocitrate Dehydrogenase-1 (IDH1) is commonly mutated in lower-grade diffuse gliomas. The IDH1R132H mutation is an important diagnostic tool for tumor diagnosis and prognosis; however, its role in glioma development, and its impact on response to therapy, is not fully understood. We developed a murine model of proneural IDH1R132H-mutated glioma that shows elevated production of 2-hydroxyglutarate (2-HG) and increased trimethylation of lysine residue K27 on histone H3 (H3K27me3) compared to IDH1 wild-type tumors. We found that using Tazemetostat to inhibit the methyltransferase for H3K27, Enhancer of Zeste 2 (EZH2), reduced H3K27me3 levels and increased acetylation on H3K27. We also found that, although the histone deacetylase inhibitor (HDACi) Panobinostat was less cytotoxic in IDH1R132H-mutated cells (either isolated from murine glioma or oligodendrocyte progenitor cells infected in vitro with a retrovirus expressing IDH1R132H) compared to IDH1-wild-type cells, combination treatment with Tazemetostat is synergistic in both mutant and wild-type models. These findings indicate a novel therapeutic strategy for IDH1-mutated gliomas that targets the specific epigenetic alteration in these tumors.
Document Type: article in journal/newspaper
Language: unknown
Relation: qt3820t8bm; https://escholarship.org/uc/item/3820t8bm
DOI: 10.3390/cells13030219
Availability: https://escholarship.org/uc/item/3820t8bm; https://doi.org/10.3390/cells13030219
Rights: public
Accession Number: edsbas.F046504B
Database: BASE