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@ARTICLE{Haag:167358,
author = {D. Haag$^*$ and N. Mack$^*$ and P. Benites Goncalves da
Silva$^*$ and B. Statz$^*$ and J. Clark$^*$ and K. Tanabe
and T. Sharma$^*$ and N. Jäger$^*$ and D. T. W. Jones$^*$
and D. Kawauchi$^*$ and M. Wernig and S. Pfister$^*$},
title = {{H}3.3-{K}27{M} drives neural stem cell-specific
gliomagenesis in a human i{PSC}-derived model.},
journal = {Cancer cell},
volume = {39},
number = {3},
issn = {1535-6108},
address = {New York, NY},
publisher = {Elsevier},
reportid = {DKFZ-2021-00304},
pages = {407-422.e13},
year = {2021},
note = {#EA:B062#LA:B062#2021 Mar 8;39(3):407-422.e13},
abstract = {Diffuse intrinsic pontine glioma (DIPG) is an aggressive
childhood tumor of the brainstem with currently no curative
treatment available. The vast majority of DIPGs carry a
histone H3 mutation leading to a lysine 27-to-methionine
exchange (H3K27M). We engineered human induced pluripotent
stem cells (iPSCs) to carry an inducible H3.3-K27M allele in
the endogenous locus and studied the effects of the mutation
in different disease-relevant neural cell types. H3.3-K27M
upregulated bivalent promoter-associated developmental
genes, producing diverse outcomes in different cell types.
While being fatal for iPSCs, H3.3-K27M increased
proliferation in neural stem cells (NSCs) and to a lesser
extent in oligodendrocyte progenitor cells (OPCs). Only NSCs
gave rise to tumors upon induction of H3.3-K27M and TP53
inactivation in an orthotopic xenograft model recapitulating
human DIPGs. In NSCs, H3.3-K27M leads to maintained
expression of stemness and proliferative genes and a
premature activation of OPC programs that together may cause
tumor initiation.},
keywords = {DIPG (Other) / H3.3-K27M (Other) / H3K27me3 (Other) /
H3K4me3 (Other) / NSC (Other) / OPC (Other) / bivalent
chromatin (Other) / glioma (Other) / iPSC (Other) /
orthotopic xenograft (Other)},
cin = {B062 / HD01 / B360},
ddc = {610},
cid = {I:(DE-He78)B062-20160331 / I:(DE-He78)HD01-20160331 /
I:(DE-He78)B360-20160331},
pnm = {312 - Funktionelle und strukturelle Genomforschung
(POF4-312)},
pid = {G:(DE-HGF)POF4-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:33545065},
doi = {10.1016/j.ccell.2021.01.005},
url = {https://inrepo02.dkfz.de/record/167358},
}