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@ARTICLE{Heinst:292539,
author = {L. Heinst and K. Lee$^*$ and R. Berthold and I. Isfort and
S. Wosnig and A. Kuntze and S. Hafner and B. Altvater and C.
Rössig and P. Åman and E. Wardelmann and C. Scholl$^*$ and
W. Hartmann and S. Fröhling$^*$ and M. Trautmann},
title = {{E}xploiting {WEE}1 kinase activity as
{FUS}::{DDIT}3-dependent therapeutic vulnerability in myxoid
liposarcoma.},
journal = {Clinical cancer research},
volume = {30},
number = {21},
issn = {1078-0432},
address = {Philadelphia, Pa. [u.a.]},
publisher = {AACR},
reportid = {DKFZ-2024-01782},
pages = {4974-4986},
year = {2024},
note = {2024 Nov 1;30(21):4974-4986},
abstract = {The pathognomonic FUS::DDIT3 fusion protein drives myxoid
liposarcoma (MLS) tumorigenesis via aberrant transcriptional
activation of oncogenic signaling. Since FUS::DDIT3 has so
far not been pharmacologically tractable to selectively
target MLS cells, this study investigated the functional
role of the cell cycle regulator WEE1 as novel
FUS::DDIT3‑dependent therapeutic vulnerability in
MLS.Immunohistochemical evaluation of the cell cycle
regulator WEE1 was performed in a large cohort of MLS
specimens. FUS::DDIT3 dependency and biological function of
the G1/S cell cycle checkpoint were analyzed in a
mesenchymal stem cell model and liposarcoma cell lines in
vitro. WEE1 activity was modulated by RNAi‑mediated
knockdown and the small molecule inhibitor MK-1775
(Adavosertib). An established MLS cell line-based chicken
chorioallantoic membrane model was employed for in vivo
confirmation.We demonstrate that enhanced WEE1 pathway
activity represents a hallmark of FUS::DDIT3‑expressing
cell lines as well as MLS tissue specimens and that WEE1 is
required for MLS cellular survival in vitro and in vivo.
Pharmacologic inhibition of WEE1 activity results in DNA
damage accumulation and cell cycle progression forcing cells
to undergo apoptotic cell death. In addition, our results
uncover FUS::DDIT3-dependent WEE1 expression as an oncogenic
survival mechanism to tolerate high proliferation and
resulting replication stress in MLS. Fusion protein-driven
G1/S cell cycle checkpoint deregulation via overactive
Cyclin E/CDK2 complexes thereby contributes to enhanced WEE1
inhibitor sensitivity in MLS.Our preclinical study
identifies WEE1-mediated replication stress tolerance as
molecular vulnerability in FUS::DDIT3-driven MLS
tumorigenesis that could represent a novel target for
therapeutic intervention.},
cin = {B340 / HD01 / B290},
ddc = {610},
cid = {I:(DE-He78)B340-20160331 / I:(DE-He78)HD01-20160331 /
I:(DE-He78)B290-20160331},
pnm = {312 - Funktionelle und strukturelle Genomforschung
(POF4-312)},
pid = {G:(DE-HGF)POF4-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:39207225},
doi = {10.1158/1078-0432.CCR-24-1152},
url = {https://inrepo02.dkfz.de/record/292539},
}