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@ARTICLE{Treisman:147736,
author = {D. M. Treisman and Y. Li and B. R. Pierce and C. Li and A.
P. Chervenak and G. J. Tomasek and G. Lozano and X. Zheng
and M. Kool$^*$ and Y. Zhu},
title = {{S}ox2+ cells in {S}onic {H}edgehog-subtype medulloblastoma
resist p53-mediated cell-cycle arrest response and drive
therapy-induced recurrence.},
journal = {Neuro-oncology advances},
volume = {1},
number = {1},
issn = {2632-2498},
address = {Oxford},
publisher = {Oxford University Press},
reportid = {DKFZ-2019-02713},
pages = {vdz027},
year = {2019},
note = {Neuro-Oncology Advances () = 2632-2498 (import from
CrossRef, PubMed, )E-ISSN(s): 2632-2498},
abstract = {High-intensity therapy effectively treats most TP53
wild-type (TP53-WT) Sonic Hedgehog-subgroup medulloblastomas
(SHH-MBs), but often cause long-term deleterious
neurotoxicities in children. Recent clinical trials
investigating reduction/de-escalation of therapy for TP53-WT
SHH-MBs caused poor overall survival. Here, we investigated
whether reduced levels of p53-pathway activation by
low-intensity therapy potentially contribute to diminished
therapeutic efficacy.Using mouse SHH-MB models with
different p53 activities, we investigated therapeutic
efficacy by activating p53-mediated cell-cycle arrest versus
p53-mediated apoptosis on radiation-induced recurrence.Upon
radiation treatment, p53WT-mediated apoptosis was sufficient
to eliminate all SHH-MB cells, including Sox2+ cells. The
same treatment eliminated most Sox2- bulk tumor cells in
SHH-MBs harboring p53R172P, an apoptosis-defective allele
with cell-cycle arrest activity, via inducing robust
neuronal differentiation. Rare quiescent Sox2+ cells
survived radiation-enhanced p53R172P activation and entered
a proliferative state, regenerating tumors. Transcriptomes
of Sox2+ cells resembled quiescent Nestin-expressing
progenitors in the developing cerebellum, expressing Olig2
known to suppress p53 and p21 expression. Importantly, high
SOX2 expression is associated with poor survival of all four
SHH-MB subgroups, independent of TP53 mutational
status.Quiescent Sox2+ cells are efficiently eliminated by
p53-mediated apoptosis, but not cell-cycle arrest and
differentiation. Their survival contributes to tumor
recurrence due to insufficient p53-pathway activation.},
cin = {B062 / L101},
ddc = {610},
cid = {I:(DE-He78)B062-20160331 / I:(DE-He78)L101-20160331},
pnm = {312 - Functional and structural genomics (POF3-312)},
pid = {G:(DE-HGF)POF3-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:31763624},
pmc = {pmc:PMC6860004},
doi = {10.1093/noajnl/vdz027},
url = {https://inrepo02.dkfz.de/record/147736},
}