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@ARTICLE{Phillips:294336,
author = {E. Phillips$^*$ and S. van Enk$^*$ and S. Kildgaard and S.
Schlue$^*$ and M. Göttmann$^*$ and L. V. Jennings$^*$ and
F. Bethke$^*$ and G. Müller$^*$ and C. Herold-Mende and D.
Pastor-Flores$^*$ and M. Schneider$^*$ and D. Helm$^*$ and
T. Ostenfeld Larsen and V. Goidts$^*$},
title = {{M}alformin {C} preferentially kills glioblastoma stem-like
cells via concerted induction of proteotoxic stress and
autophagic flux blockade.},
journal = {Molecular oncology},
volume = {19},
number = {3},
issn = {1574-7891},
address = {Hoboken, NJ},
publisher = {John Wiley $\&$ Sons, Inc.},
reportid = {DKFZ-2024-02169},
pages = {785-807},
year = {2025},
note = {#EA:B067#LA:B067# / 2025 Mar;19(3):785-807},
abstract = {Glioblastoma is a highly aggressive brain tumor for which
there is no cure. The dire prognosis of this disease is
largely attributable to a high level of heterogeneity,
including the presence of a subpopulation of
tumor-initiating glioblastoma stem-like cells (GSCs), which
are refractory to chemo- and radiotherapy. Here, in an
unbiased marine-derived fungal extract screen, together with
bioguided dereplication based on high-resolution mass
spectrometry, we identified malformin C to preferentially
induce cell death in patient-derived GSCs and explore the
potential of this cyclic peptide as a therapeutic agent for
glioblastoma. Malformin C significantly reduced tumor growth
in an in vivo xenograft model of glioblastoma. Using
transcriptomics and chemoproteomics, we found that malformin
C binds to many proteins, leading to their aggregation, and
rapidly induces the unfolded protein response, including
autophagy, in GSCs. Crucially, chemical inhibition of
translation using cycloheximide rescued malformin C-induced
cell death in GSCs, demonstrating that the proteotoxic
effect of the compound is necessary for its cytotoxicity. At
the same time, malformin C appears to accumulate in
lysosomes, disrupting autophagic flux, and driving cells to
death. Supporting this, malformin C synergizes with
chloroquine, an inhibitor of autophagy. Strikingly, we
observed that autophagic flux is differentially regulated in
GSCs compared with normal astrocytes. The sensitivity of
GSCs to malformin C highlights the relevance of proteostasis
and autophagy as a therapeutic vulnerability in
glioblastoma.},
keywords = {autophagy (Other) / cancer (Other) / compound screen
(Other) / glioblastoma (Other) / proteostasis (Other) / stem
cells (Other) / unfolded protein response (Other)},
cin = {B067 / A160 / W120},
ddc = {610},
cid = {I:(DE-He78)B067-20160331 / I:(DE-He78)A160-20160331 /
I:(DE-He78)W120-20160331},
pnm = {312 - Funktionelle und strukturelle Genomforschung
(POF4-312)},
pid = {G:(DE-HGF)POF4-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:39462997},
doi = {10.1002/1878-0261.13756},
url = {https://inrepo02.dkfz.de/record/294336},
}