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Article

The MALAT1-EZH2 axis regulates PRC2 activity and promotes the mesenchymal phenotype in pediatric atypical teratoid/rhabdoid tumors.

Journal of neuro-oncology - 31 Mar 2026

Gurbuz Melisa, Tekin Cagla, Ercelik Melis, Koc Sevin Avsar, Kockar Feray, Eser Pınar, Taskapilioglu Mevlut Ozgur, Tezcan Gulcin, Erbaykent Burcu, Bekar Ahmet, Kocaeli Hasan, Ozsen Mine, Karabaglı Pınar, Karabaglı Hakan, Bayrak Buşra Yaprak, Etus Volkan, Tunca Berrin

Abstract excerpt

BACKGROUND: Atypical teratoid/rhabdoid tumors (AT/RT) are aggressive pediatric CNS malignancies characterized by SMARCB1 loss, which leads to the dysregulated expression of Enhancer of Zeste Homolog 2 (EZH2), a key catalytic component of the Polycomb Repressive Complex 2 (PRC2). This dysregulation results in aberrant trimethylation of histone H3 at lysine 27 (H3K27me3), driving tumor progression. While EZH2...

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Identifiers

PMID: 41915306
DOI: 10.1007/s11060-026-05539-x
PMCID: PMC13038470

Genes

CDH2
ZEB1
SMARCB1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026.

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