The dystroglycan receptor maintains glioma stem cells in the vascular niche.


Journal

Acta neuropathologica
ISSN: 1432-0533
Titre abrégé: Acta Neuropathol
Pays: Germany
ID NLM: 0412041

Informations de publication

Date de publication:
12 2019
Historique:
received: 09 05 2019
accepted: 22 08 2019
revised: 21 08 2019
pubmed: 30 8 2019
medline: 4 9 2020
entrez: 30 8 2019
Statut: ppublish

Résumé

Glioblastomas (GBMs) are malignant central nervous system (CNS) neoplasms with a very poor prognosis. They display cellular hierarchies containing self-renewing tumourigenic glioma stem cells (GSCs) in a complex heterogeneous microenvironment. One proposed GSC niche is the extracellular matrix (ECM)-rich perivascular bed of the tumour. Here, we report that the ECM binding dystroglycan (DG) receptor is expressed and functionally glycosylated on GSCs residing in the perivascular niche. Glycosylated αDG is highly expressed and functional on the most aggressive mesenchymal-like (MES-like) GBM tumour compartment. Furthermore, we found that DG acts to maintain an MES-like state via tight control of MAPK activation. Antibody-based blockade of αDG induces robust ERK-mediated differentiation leading to reduced GSC potential. DG was shown to be required for tumour initiation in MES-like GBM, with constitutive loss significantly delaying or preventing tumourigenic potential in-vivo. These findings reveal a central role of the DG receptor, not only as a structural element, but also as a critical factor promoting MES-like GBM and the maintenance of GSCs residing in the perivascular niche.

Identifiants

pubmed: 31463571
doi: 10.1007/s00401-019-02069-x
pii: 10.1007/s00401-019-02069-x
pmc: PMC6851226
doi:

Substances chimiques

Dystroglycans 146888-27-9
Extracellular Signal-Regulated MAP Kinases EC 2.7.11.24

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

1033-1052

Subventions

Organisme : National Health and Medical Research Council (NHMRC) - Australia
ID : I.D.1078893
Pays : International
Organisme : Paul D. Wellstone Muscular Dystrophy Cooperative Research Center
ID : 1U54NS053672
Pays : International

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Auteurs

Bryan W Day (BW)

Department of Cell and Molecular Biology, Sid Faithfull Brain Cancer Laboratory, QIMR Berghofer Medical Research Institute, Brisbane, QLD, 4006, Australia. Bryan.Day@qimrberghofer.edu.au.
Faculty of Health, Queensland University of Technology, Brisbane, 4059, Australia. Bryan.Day@qimrberghofer.edu.au.
Faculty of Medicine, The University of Queensland, Brisbane, 4072, Australia. Bryan.Day@qimrberghofer.edu.au.

Justin D Lathia (JD)

Cleveland Clinic, Lerner College of Medicine, Case Western Reserve University, Cleveland, OH, 44195, USA.

Zara C Bruce (ZC)

Department of Cell and Molecular Biology, Sid Faithfull Brain Cancer Laboratory, QIMR Berghofer Medical Research Institute, Brisbane, QLD, 4006, Australia.

Rochelle C J D'Souza (RCJ)

Department of Cell and Molecular Biology, Sid Faithfull Brain Cancer Laboratory, QIMR Berghofer Medical Research Institute, Brisbane, QLD, 4006, Australia.

Ulrich Baumgartner (U)

Department of Cell and Molecular Biology, Sid Faithfull Brain Cancer Laboratory, QIMR Berghofer Medical Research Institute, Brisbane, QLD, 4006, Australia.

Kathleen S Ensbey (KS)

Department of Cell and Molecular Biology, Sid Faithfull Brain Cancer Laboratory, QIMR Berghofer Medical Research Institute, Brisbane, QLD, 4006, Australia.

Yi Chieh Lim (YC)

Department of Cell and Molecular Biology, Sid Faithfull Brain Cancer Laboratory, QIMR Berghofer Medical Research Institute, Brisbane, QLD, 4006, Australia.

Brett W Stringer (BW)

Department of Cell and Molecular Biology, Sid Faithfull Brain Cancer Laboratory, QIMR Berghofer Medical Research Institute, Brisbane, QLD, 4006, Australia.

Seçkin Akgül (S)

Department of Cell and Molecular Biology, Sid Faithfull Brain Cancer Laboratory, QIMR Berghofer Medical Research Institute, Brisbane, QLD, 4006, Australia.

Carolin Offenhäuser (C)

Department of Cell and Molecular Biology, Sid Faithfull Brain Cancer Laboratory, QIMR Berghofer Medical Research Institute, Brisbane, QLD, 4006, Australia.

Yuchen Li (Y)

Department of Cell and Molecular Biology, Sid Faithfull Brain Cancer Laboratory, QIMR Berghofer Medical Research Institute, Brisbane, QLD, 4006, Australia.

Paul R Jamieson (PR)

Department of Cell and Molecular Biology, Sid Faithfull Brain Cancer Laboratory, QIMR Berghofer Medical Research Institute, Brisbane, QLD, 4006, Australia.

Fiona M Smith (FM)

Department of Cell and Molecular Biology, Sid Faithfull Brain Cancer Laboratory, QIMR Berghofer Medical Research Institute, Brisbane, QLD, 4006, Australia.

Courtney L R Jurd (CLR)

Department of Cell and Molecular Biology, Sid Faithfull Brain Cancer Laboratory, QIMR Berghofer Medical Research Institute, Brisbane, QLD, 4006, Australia.

Thomas Robertson (T)

Royal Brisbane and Women's Hospital, Brisbane, QLD, 4006, Australia.

Po-Ling Inglis (PL)

Royal Brisbane and Women's Hospital, Brisbane, QLD, 4006, Australia.

Zarnie Lwin (Z)

Royal Brisbane and Women's Hospital, Brisbane, QLD, 4006, Australia.

Rosalind L Jeffree (RL)

Royal Brisbane and Women's Hospital, Brisbane, QLD, 4006, Australia.

Terrance G Johns (TG)

Telethon Kids Institute, Perth, WA, 6009, Australia.

Krishna P L Bhat (KPL)

Department of Translational Molecular Pathology, The University of Texas, MD Anderson Cancer Center, Houston, TX, 77030, USA.

Jeremy N Rich (JN)

Medicine Department, University of California, La Jolla, San Diego, CA, 92093-0021, USA.

Kevin P Campbell (KP)

Department of Molecular Physiology and Biophysics, Roy J. and Lucille A. Carver College of Medicine, Howard Hughes Medical Institute, University of Iowa, Iowa City, IA, 52242, USA.
Department of Neurology, Roy J. and Lucille A. Carver College of Medicine, Howard Hughes Medical Institute, University of Iowa, Iowa City, IA, 52242, USA.

Andrew W Boyd (AW)

Department of Cell and Molecular Biology, Sid Faithfull Brain Cancer Laboratory, QIMR Berghofer Medical Research Institute, Brisbane, QLD, 4006, Australia.
Faculty of Medicine, The University of Queensland, Brisbane, 4072, Australia.

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