Logo image
The dystroglycan receptor maintains glioma stem cells in the vascular niche
Journal article   Open access   Peer reviewed

The dystroglycan receptor maintains glioma stem cells in the vascular niche

Bryan W Day, Justin D Lathia, Zara C Bruce, Rochelle C. J D’Souza, Ulrich Baumgartner, Kathleen S Ensbey, Yi Chieh Lim, Brett W Stringer, Seçkin Akgül, Carolin Offenhäuser, …
Acta neuropathologica, Vol.138(6), pp.1033-1052
2019
DOI: 10.1007/s00401-019-02069-x
PMCID: PMC6851226
PMID: 31463571
url
https://doi.org/10.1007/s00401-019-02069-xView
Published (Version of record) Open Access

Abstract

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.
Glioma stem cell (GSC) commitment Glioblastoma (GBM) MES-like GBM Original Paper Dystroglycan (DG) Perivascular niche MAPK signalling EphA3 Integrin-α6

Details

Logo image