Malformin C preferentially kills glioblastoma stem‐like cells via concerted induction of proteotoxic stress and autophagic flux blockade

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‐ a...

Full description

Saved in:
Bibliographic Details
Main Authors: Emma Phillips, Sizèd vanEnk, Sara Kildgaard, Silja Schlue, Mona Göttmann, Victoria Jennings, Frederic Bethke, Gabriele Müller, Christel Herold‐Mende, Daniel Pastor‐Flores, Martin Schneider, Dominic Helm, Thomas Ostenfeld Larsen, Violaine Goidts
Format: Article
Language:English
Published: Wiley 2025-03-01
Series:Molecular Oncology
Subjects:
Online Access:https://doi.org/10.1002/1878-0261.13756
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1850028901816860672
author Emma Phillips
Sizèd vanEnk
Sara Kildgaard
Silja Schlue
Mona Göttmann
Victoria Jennings
Frederic Bethke
Gabriele Müller
Christel Herold‐Mende
Daniel Pastor‐Flores
Martin Schneider
Dominic Helm
Thomas Ostenfeld Larsen
Violaine Goidts
author_facet Emma Phillips
Sizèd vanEnk
Sara Kildgaard
Silja Schlue
Mona Göttmann
Victoria Jennings
Frederic Bethke
Gabriele Müller
Christel Herold‐Mende
Daniel Pastor‐Flores
Martin Schneider
Dominic Helm
Thomas Ostenfeld Larsen
Violaine Goidts
author_sort Emma Phillips
collection DOAJ
description 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.
format Article
id doaj-art-0ec555fe0b3a42eeb8581e43b438aae2
institution DOAJ
issn 1574-7891
1878-0261
language English
publishDate 2025-03-01
publisher Wiley
record_format Article
series Molecular Oncology
spelling doaj-art-0ec555fe0b3a42eeb8581e43b438aae22025-08-20T02:59:42ZengWileyMolecular Oncology1574-78911878-02612025-03-0119378580710.1002/1878-0261.13756Malformin C preferentially kills glioblastoma stem‐like cells via concerted induction of proteotoxic stress and autophagic flux blockadeEmma Phillips0Sizèd vanEnk1Sara Kildgaard2Silja Schlue3Mona Göttmann4Victoria Jennings5Frederic Bethke6Gabriele Müller7Christel Herold‐Mende8Daniel Pastor‐Flores9Martin Schneider10Dominic Helm11Thomas Ostenfeld Larsen12Violaine Goidts13Junior Research Group “Brain Tumor Translational Targets” German Cancer Research Center (DKFZ) Heidelberg GermanyJunior Research Group “Brain Tumor Translational Targets” German Cancer Research Center (DKFZ) Heidelberg GermanyDepartment of Biotechnology and Biomedicine, Section for Microbial and Chemical Ecology, Natural Product Discovery Technical University of Denmark Copenhagen DenmarkJunior Research Group “Brain Tumor Translational Targets” German Cancer Research Center (DKFZ) Heidelberg GermanyJunior Research Group “Brain Tumor Translational Targets” German Cancer Research Center (DKFZ) Heidelberg GermanyJunior Research Group “Brain Tumor Translational Targets” German Cancer Research Center (DKFZ) Heidelberg GermanyJunior Research Group “Brain Tumor Translational Targets” German Cancer Research Center (DKFZ) Heidelberg GermanyJunior Research Group “Brain Tumor Translational Targets” German Cancer Research Center (DKFZ) Heidelberg GermanyDivision of Neurosurgical Research, Department of Neurosurgery University Hospital Heidelberg GermanyDivision of Redox Regulation German Cancer Research Center (DKFZ) Heidelberg GermanyProteomics Core Facility German Cancer Research Center (DKFZ) Heidelberg GermanyProteomics Core Facility German Cancer Research Center (DKFZ) Heidelberg GermanyDepartment of Biotechnology and Biomedicine, Section for Microbial and Chemical Ecology, Natural Product Discovery Technical University of Denmark Copenhagen DenmarkJunior Research Group “Brain Tumor Translational Targets” German Cancer Research Center (DKFZ) Heidelberg GermanyGlioblastoma 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.https://doi.org/10.1002/1878-0261.13756autophagycancercompound screenglioblastomaproteostasisstem cells
spellingShingle Emma Phillips
Sizèd vanEnk
Sara Kildgaard
Silja Schlue
Mona Göttmann
Victoria Jennings
Frederic Bethke
Gabriele Müller
Christel Herold‐Mende
Daniel Pastor‐Flores
Martin Schneider
Dominic Helm
Thomas Ostenfeld Larsen
Violaine Goidts
Malformin C preferentially kills glioblastoma stem‐like cells via concerted induction of proteotoxic stress and autophagic flux blockade
Molecular Oncology
autophagy
cancer
compound screen
glioblastoma
proteostasis
stem cells
title Malformin C preferentially kills glioblastoma stem‐like cells via concerted induction of proteotoxic stress and autophagic flux blockade
title_full Malformin C preferentially kills glioblastoma stem‐like cells via concerted induction of proteotoxic stress and autophagic flux blockade
title_fullStr Malformin C preferentially kills glioblastoma stem‐like cells via concerted induction of proteotoxic stress and autophagic flux blockade
title_full_unstemmed Malformin C preferentially kills glioblastoma stem‐like cells via concerted induction of proteotoxic stress and autophagic flux blockade
title_short Malformin C preferentially kills glioblastoma stem‐like cells via concerted induction of proteotoxic stress and autophagic flux blockade
title_sort malformin c preferentially kills glioblastoma stem like cells via concerted induction of proteotoxic stress and autophagic flux blockade
topic autophagy
cancer
compound screen
glioblastoma
proteostasis
stem cells
url https://doi.org/10.1002/1878-0261.13756
work_keys_str_mv AT emmaphillips malformincpreferentiallykillsglioblastomastemlikecellsviaconcertedinductionofproteotoxicstressandautophagicfluxblockade
AT sizedvanenk malformincpreferentiallykillsglioblastomastemlikecellsviaconcertedinductionofproteotoxicstressandautophagicfluxblockade
AT sarakildgaard malformincpreferentiallykillsglioblastomastemlikecellsviaconcertedinductionofproteotoxicstressandautophagicfluxblockade
AT siljaschlue malformincpreferentiallykillsglioblastomastemlikecellsviaconcertedinductionofproteotoxicstressandautophagicfluxblockade
AT monagottmann malformincpreferentiallykillsglioblastomastemlikecellsviaconcertedinductionofproteotoxicstressandautophagicfluxblockade
AT victoriajennings malformincpreferentiallykillsglioblastomastemlikecellsviaconcertedinductionofproteotoxicstressandautophagicfluxblockade
AT fredericbethke malformincpreferentiallykillsglioblastomastemlikecellsviaconcertedinductionofproteotoxicstressandautophagicfluxblockade
AT gabrielemuller malformincpreferentiallykillsglioblastomastemlikecellsviaconcertedinductionofproteotoxicstressandautophagicfluxblockade
AT christelheroldmende malformincpreferentiallykillsglioblastomastemlikecellsviaconcertedinductionofproteotoxicstressandautophagicfluxblockade
AT danielpastorflores malformincpreferentiallykillsglioblastomastemlikecellsviaconcertedinductionofproteotoxicstressandautophagicfluxblockade
AT martinschneider malformincpreferentiallykillsglioblastomastemlikecellsviaconcertedinductionofproteotoxicstressandautophagicfluxblockade
AT dominichelm malformincpreferentiallykillsglioblastomastemlikecellsviaconcertedinductionofproteotoxicstressandautophagicfluxblockade
AT thomasostenfeldlarsen malformincpreferentiallykillsglioblastomastemlikecellsviaconcertedinductionofproteotoxicstressandautophagicfluxblockade
AT violainegoidts malformincpreferentiallykillsglioblastomastemlikecellsviaconcertedinductionofproteotoxicstressandautophagicfluxblockade