TY  - JOUR
AU  - Zheng, Tuyu
AU  - Ghasemi, David R
AU  - Okonechnikov, Konstantin
AU  - Korshunov, Andrey
AU  - Sill, Martin
AU  - Maass, Kendra K
AU  - Benites Goncalves da Silva, Patricia
AU  - Ryzhova, Marina
AU  - Gojo, Johannes
AU  - Stichel, Damian
AU  - Arabzade, Amir
AU  - Kupp, Robert
AU  - Benzel, Julia
AU  - Taya, Shinichiro
AU  - Adachi, Toma
AU  - Shiraishi, Ryo
AU  - Gerber, Nicolas U
AU  - Sturm, Dominik
AU  - Ecker, Jonas
AU  - Sievers, Philipp
AU  - Selt, Florian
AU  - Chapman, Rebecca
AU  - Haberler, Christine
AU  - Figarella-Branger, Dominique
AU  - Reifenberger, Guido
AU  - Fleischhack, Gudrun
AU  - Rutkowski, Stefan
AU  - Donson, Andrew M
AU  - Ramaswamy, Vijay
AU  - Capper, David
AU  - Ellison, David W
AU  - Herold-Mende, Christel C
AU  - Schuller, Ulrich
AU  - Brandner, Sebastian
AU  - Hernaiz Driever, Pablo
AU  - Kros, Johan M
AU  - Snuderl, Matija
AU  - Milde, Till
AU  - Grundy, Richard G
AU  - Hoshino, Mikio
AU  - Mack, Stephen C
AU  - Gilbertson, Richard J
AU  - Jones, David T W
AU  - Kool, Marcel
AU  - von Deimling, Andreas
AU  - Pfister, Stefan M
AU  - Sahm, Felix
AU  - Kawauchi, Daisuke
AU  - Pajtler, Kristian
TI  - Cross-species genomics reveals oncogenic dependencies in ZFTA/C11orf95 fusion-positive supratentorial ependymomas.
JO  - Cancer discovery
VL  - 11
IS  - 9
SN  - 2159-8290
CY  - Philadelphia, Pa.
M1  - DKFZ-2021-00917
SP  - 2230-2247
PY  - 2021
N1  - #EA:B062#LA:B062#B300# /2021 Sep;11(9):2230-2247
AB  - Molecular groups of supratentorial ependymomas comprise tumors with ZFTA-RELA or YAP1-involving fusions and fusion-negative subependymoma. However, occasionally supratentorial ependymomas cannot be readily assigned to any of these groups due to lack of detection of a typical fusion and/or ambiguous DNA methylation-based classification. An unbiased approach with a cohort of unprecedented size revealed distinct methylation clusters composed of tumors with ependymal but also various other histological features containing alternative translocations that shared ZFTA as a partner gene. Somatic overexpression of ZFTA-associated fusion genes in the developing cerebral cortex is capable of inducing tumor formation in vivo, and cross-species comparative analyses identified GLI2 as a key downstream regulator of tumorigenesis in all tumors. Targeting GLI2 with arsenic trioxide caused extended survival of tumor-bearing animals, indicating a potential therapeutic vulnerability in ZFTA fusion-positive tumors.
LB  - PUB:(DE-HGF)16
C6  - pmid:33879448
DO  - DOI:10.1158/2159-8290.CD-20-0963
UR  - https://inrepo02.dkfz.de/record/168443
ER  -