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

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

  • By

  • Melisa Gurbuz

  • Cagla Tekin

  • Melis Ercelik

  • Sevin Avsar Koc

  • Feray Kockar

  • Pınar Eser

  • Mevlut Ozgur Taskapilioglu

  • Gulcin Tezcan

  • Burcu Erbaykent

  • Ahmet Bekar

  • Hasan Kocaeli

  • Mine Ozsen

  • Pınar Karabaglı

  • Hakan Karabaglı

  • Buşra Yaprak Bayrak

  • Volkan Etus

  • Berrin Tunca

  • March 31, 2026

  • 0 min

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  • 1

    Atypical teratoid/rhabdoid tumors (AT/RTs) are aggressive pediatric CNS malignancies primarily affecting children under three years old.

  • 2

    SMARCB1 loss is a unifying driver of AT/RTs, leading to dysregulation of the EZH2/PRC2 pathway and promoting oncogenesis.

  • 3

    lncRNAs like MALAT1 are dysregulated in AT/RTs and can modulate EZH2 activity, contributing to tumor progression and therapy resistance.

  • 4

    The EZH2 inhibitor tazemetostat has shown limited clinical success in treating SMARCB1-deficient tumors, highlighting the need for alternative strategies.

  • 5

    Targeting lncRNAs may suppress tumor aggressiveness and mesenchymal features in AT/RTs, offering a potential therapeutic avenue.

Original Source(s)

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

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

  • March 31, 2026

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