ETMR stem-like state and chemo-resistance are supported by perivascular cells at single-cell resolution

Abstract Embryonal tumor with multilayered rosettes (ETMR) is a lethal embryonal brain tumor entity. To investigate the intratumoral heterogeneity and cellular communication in the tumor microenvironment (TME), we analyze in this work single-cell RNA sequencing of about 250,000 cells of primary huma...

Full description

Saved in:
Bibliographic Details
Main Authors: Flavia W. de Faria, Nicole C. Riedel, Daniel Münter, Marta Interlandi, Carolin Göbel, Lea Altendorf, Mathis Richter, Viktoria Melcher, Christian Thomas, Rajanya Roy, Melanie Schoof, Ivan Bedzhov, Natalia Moreno, Monika Graf, Marc Hotfilder, Dörthe Holdhof, Wolfgang Hartmann, Ann-Katrin Bruns, Angela Brentrup, Friederike Liesche-Starnecker, Bruno Maerkl, Sarah Sandmann, Julian Varghese, Martin Dugas, Pedro H. Pinto, Sebastian T. Balbach, I-Na Lu, Claudia Rossig, Oliver Soehnlein, Aysegül Canak, Martin Ebinger, Martin Schuhmann, Jens Schittenhelm, Michael F. Frühwald, Ulrich Schüller, Thomas K. Albert, Carolin Walter, Jan M. Bruder, Kornelius Kerl
Format: Article
Language:English
Published: Nature Portfolio 2025-06-01
Series:Nature Communications
Online Access:https://doi.org/10.1038/s41467-025-60442-9
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1850109860714119168
author Flavia W. de Faria
Nicole C. Riedel
Daniel Münter
Marta Interlandi
Carolin Göbel
Lea Altendorf
Mathis Richter
Viktoria Melcher
Christian Thomas
Rajanya Roy
Melanie Schoof
Ivan Bedzhov
Natalia Moreno
Monika Graf
Marc Hotfilder
Dörthe Holdhof
Wolfgang Hartmann
Ann-Katrin Bruns
Angela Brentrup
Friederike Liesche-Starnecker
Bruno Maerkl
Sarah Sandmann
Julian Varghese
Martin Dugas
Pedro H. Pinto
Sebastian T. Balbach
I-Na Lu
Claudia Rossig
Oliver Soehnlein
Aysegül Canak
Martin Ebinger
Martin Schuhmann
Jens Schittenhelm
Michael F. Frühwald
Ulrich Schüller
Thomas K. Albert
Carolin Walter
Jan M. Bruder
Kornelius Kerl
author_facet Flavia W. de Faria
Nicole C. Riedel
Daniel Münter
Marta Interlandi
Carolin Göbel
Lea Altendorf
Mathis Richter
Viktoria Melcher
Christian Thomas
Rajanya Roy
Melanie Schoof
Ivan Bedzhov
Natalia Moreno
Monika Graf
Marc Hotfilder
Dörthe Holdhof
Wolfgang Hartmann
Ann-Katrin Bruns
Angela Brentrup
Friederike Liesche-Starnecker
Bruno Maerkl
Sarah Sandmann
Julian Varghese
Martin Dugas
Pedro H. Pinto
Sebastian T. Balbach
I-Na Lu
Claudia Rossig
Oliver Soehnlein
Aysegül Canak
Martin Ebinger
Martin Schuhmann
Jens Schittenhelm
Michael F. Frühwald
Ulrich Schüller
Thomas K. Albert
Carolin Walter
Jan M. Bruder
Kornelius Kerl
author_sort Flavia W. de Faria
collection DOAJ
description Abstract Embryonal tumor with multilayered rosettes (ETMR) is a lethal embryonal brain tumor entity. To investigate the intratumoral heterogeneity and cellular communication in the tumor microenvironment (TME), we analyze in this work single-cell RNA sequencing of about 250,000 cells of primary human and murine ETMR, in vitro cultures, and a 3D forebrain organoid model of ETMR, supporting the main findings with immunohistochemistry and spatial transcriptomics of human tumors. We characterize three distinct malignant ETMR subpopulations - RG-like, NProg-like and NB-like - positioned within a putative neurodevelopmental hierarchy. We reveal PDGFRβ+ pericytes as key communication partners in the TME, contributing to stem cell signaling through extracellular matrix-mediated interactions with tumor cells. PDGF signaling is upregulated in chemoresistant RG-like cells in vivo and plays a role in recruiting pericytes to ETMR TME by finalizing a signaling cascade which promotes the differentiation of non-malignant radial glia cells, derived from our 3D model, into pericyte-like cells. Selective PDGFR-inhibition blocked the lineage differentiation into pericytes in vitro and reduced the tumor cell population in vivo. Targeting ETMR-pericyte interactions in the TME presents a promising therapeutic approach.
format Article
id doaj-art-549d4fe2f7db480184d2718c171c3dd6
institution OA Journals
issn 2041-1723
language English
publishDate 2025-06-01
publisher Nature Portfolio
record_format Article
series Nature Communications
spelling doaj-art-549d4fe2f7db480184d2718c171c3dd62025-08-20T02:37:58ZengNature PortfolioNature Communications2041-17232025-06-0116111910.1038/s41467-025-60442-9ETMR stem-like state and chemo-resistance are supported by perivascular cells at single-cell resolutionFlavia W. de Faria0Nicole C. Riedel1Daniel Münter2Marta Interlandi3Carolin Göbel4Lea Altendorf5Mathis Richter6Viktoria Melcher7Christian Thomas8Rajanya Roy9Melanie Schoof10Ivan Bedzhov11Natalia Moreno12Monika Graf13Marc Hotfilder14Dörthe Holdhof15Wolfgang Hartmann16Ann-Katrin Bruns17Angela Brentrup18Friederike Liesche-Starnecker19Bruno Maerkl20Sarah Sandmann21Julian Varghese22Martin Dugas23Pedro H. Pinto24Sebastian T. Balbach25I-Na Lu26Claudia Rossig27Oliver Soehnlein28Aysegül Canak29Martin Ebinger30Martin Schuhmann31Jens Schittenhelm32Michael F. Frühwald33Ulrich Schüller34Thomas K. Albert35Carolin Walter36Jan M. Bruder37Kornelius Kerl38Department of Pediatric Hematology and Oncology, University Hospital MünsterDepartment of Pediatric Hematology and Oncology, University Hospital MünsterDepartment of Pediatric Hematology and Oncology, University Hospital MünsterDepartment of Pediatric Hematology and Oncology, University Hospital MünsterDepartment of Pediatric Hematology and Oncology, University Medical Center Hamburg-EppendorfDepartment of Pediatric Hematology and Oncology, University Medical Center Hamburg-EppendorfInstitute for Experimental Pathology, Center for Molecular Biology of Inflammation, University of MünsterDepartment of Pediatric Hematology and Oncology, University Hospital MünsterInstitute of Neuropathology, University Hospital MünsterDepartment of Pediatric Hematology and Oncology, University Hospital MünsterDepartment of Pediatric Hematology and Oncology, University Medical Center Hamburg-EppendorfEmbryonic Self-Organization Research Group, Max Planck Institute for Molecular BiomedicineDepartment of Pediatric Hematology and Oncology, University Hospital MünsterDepartment of Pediatric Hematology and Oncology, University Hospital MünsterDepartment of Pediatric Hematology and Oncology, University Hospital MünsterDepartment of Pediatric Hematology and Oncology, University Medical Center Hamburg-EppendorfDivision of Translational Pathology, Gerhard-Domagk Institute of Pathology, University Hospital MünsterDepartment of Neurosurgery, University Hospital MünsterDepartment of Neurosurgery, University Hospital MünsterDepartment of Neuropathology, Pathology, Medical Faculty, University of AugsburgDepartment of Neuropathology, Pathology, Medical Faculty, University of AugsburgInstitute of Medical Informatics, Westphalian Wilhelms University MünsterInstitute of Medical Informatics, Westphalian Wilhelms University MünsterInstitute of Medical Informatics, Westphalian Wilhelms University MünsterDepartment of Pathology, Children’s Hospital of Brasilia Jose de AlencarDepartment of Pediatric Hematology and Oncology, University Hospital MünsterDepartment of Pediatric Hematology and Oncology, University Hospital MünsterDepartment of Pediatric Hematology and Oncology, University Hospital MünsterInstitute for Experimental Pathology, Center for Molecular Biology of Inflammation, University of MünsterDepartment of Hematology and Oncology, Children’s University Hospital Tübingen, and German Cancer Consortium (DKTK) TübingenDepartment of Neurosurgery, section of Pediatric Neurosurgery, University Hospital Tübingen, and German Cancer Consortium (DKTK) TübingenDepartment of Neurosurgery, section of Pediatric Neurosurgery, University Hospital Tübingen, and German Cancer Consortium (DKTK) TübingenDepartment of Neuropathology, University Medical Hospital, Eberhard Karls University TübingenSwabian Children’s Cancer Center, Pediatric and Adolescent Medicine, University Center AugsburgDepartment of Pediatric Hematology and Oncology, University Medical Center Hamburg-EppendorfDepartment of Pediatric Hematology and Oncology, University Hospital MünsterDepartment of Pediatric Hematology and Oncology, University Hospital MünsterDepartment for Cell and Developmental Biology, Max Planck Institute for molecular BiomedicineDepartment of Pediatric Hematology and Oncology, University Hospital MünsterAbstract Embryonal tumor with multilayered rosettes (ETMR) is a lethal embryonal brain tumor entity. To investigate the intratumoral heterogeneity and cellular communication in the tumor microenvironment (TME), we analyze in this work single-cell RNA sequencing of about 250,000 cells of primary human and murine ETMR, in vitro cultures, and a 3D forebrain organoid model of ETMR, supporting the main findings with immunohistochemistry and spatial transcriptomics of human tumors. We characterize three distinct malignant ETMR subpopulations - RG-like, NProg-like and NB-like - positioned within a putative neurodevelopmental hierarchy. We reveal PDGFRβ+ pericytes as key communication partners in the TME, contributing to stem cell signaling through extracellular matrix-mediated interactions with tumor cells. PDGF signaling is upregulated in chemoresistant RG-like cells in vivo and plays a role in recruiting pericytes to ETMR TME by finalizing a signaling cascade which promotes the differentiation of non-malignant radial glia cells, derived from our 3D model, into pericyte-like cells. Selective PDGFR-inhibition blocked the lineage differentiation into pericytes in vitro and reduced the tumor cell population in vivo. Targeting ETMR-pericyte interactions in the TME presents a promising therapeutic approach.https://doi.org/10.1038/s41467-025-60442-9
spellingShingle Flavia W. de Faria
Nicole C. Riedel
Daniel Münter
Marta Interlandi
Carolin Göbel
Lea Altendorf
Mathis Richter
Viktoria Melcher
Christian Thomas
Rajanya Roy
Melanie Schoof
Ivan Bedzhov
Natalia Moreno
Monika Graf
Marc Hotfilder
Dörthe Holdhof
Wolfgang Hartmann
Ann-Katrin Bruns
Angela Brentrup
Friederike Liesche-Starnecker
Bruno Maerkl
Sarah Sandmann
Julian Varghese
Martin Dugas
Pedro H. Pinto
Sebastian T. Balbach
I-Na Lu
Claudia Rossig
Oliver Soehnlein
Aysegül Canak
Martin Ebinger
Martin Schuhmann
Jens Schittenhelm
Michael F. Frühwald
Ulrich Schüller
Thomas K. Albert
Carolin Walter
Jan M. Bruder
Kornelius Kerl
ETMR stem-like state and chemo-resistance are supported by perivascular cells at single-cell resolution
Nature Communications
title ETMR stem-like state and chemo-resistance are supported by perivascular cells at single-cell resolution
title_full ETMR stem-like state and chemo-resistance are supported by perivascular cells at single-cell resolution
title_fullStr ETMR stem-like state and chemo-resistance are supported by perivascular cells at single-cell resolution
title_full_unstemmed ETMR stem-like state and chemo-resistance are supported by perivascular cells at single-cell resolution
title_short ETMR stem-like state and chemo-resistance are supported by perivascular cells at single-cell resolution
title_sort etmr stem like state and chemo resistance are supported by perivascular cells at single cell resolution
url https://doi.org/10.1038/s41467-025-60442-9
work_keys_str_mv AT flaviawdefaria etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT nicolecriedel etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT danielmunter etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT martainterlandi etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT carolingobel etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT leaaltendorf etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT mathisrichter etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT viktoriamelcher etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT christianthomas etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT rajanyaroy etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT melanieschoof etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT ivanbedzhov etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT nataliamoreno etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT monikagraf etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT marchotfilder etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT dortheholdhof etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT wolfganghartmann etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT annkatrinbruns etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT angelabrentrup etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT friederikelieschestarnecker etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT brunomaerkl etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT sarahsandmann etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT julianvarghese etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT martindugas etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT pedrohpinto etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT sebastiantbalbach etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT inalu etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT claudiarossig etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT oliversoehnlein etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT aysegulcanak etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT martinebinger etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT martinschuhmann etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT jensschittenhelm etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT michaelffruhwald etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT ulrichschuller etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT thomaskalbert etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT carolinwalter etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT janmbruder etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution
AT korneliuskerl etmrstemlikestateandchemoresistancearesupportedbyperivascularcellsatsinglecellresolution