WNT/β-catenin pathway activation in Myc immortalised cerebellar progenitor cells inhibits neuronal differentiation and generates tumours resembling medulloblastoma

Background: Medulloblastoma is the most common malignant childhood brain tumour. Aberrant activation of the WNT/β-catenin pathway occurs in approximately 25% of medulloblastomas. However, its role in medulloblastoma pathogenesis is not understood. Methods: We have developed a model of WNT/β-caten...

Full description

Bibliographic Details
Main Authors: Rogers, H.A., Sousa, S., Salto, C., Arenas, E., Coyle, B., Grundy, Richard G.
Format: Article
Published: Cancer Research UK 2012
Online Access:https://eprints.nottingham.ac.uk/2542/
_version_ 1848790812212592640
author Rogers, H.A.
Sousa, S.
Salto, C.
Arenas, E.
Coyle, B.
Grundy, Richard G.
author_facet Rogers, H.A.
Sousa, S.
Salto, C.
Arenas, E.
Coyle, B.
Grundy, Richard G.
author_sort Rogers, H.A.
building Nottingham Research Data Repository
collection Online Access
description Background: Medulloblastoma is the most common malignant childhood brain tumour. Aberrant activation of the WNT/β-catenin pathway occurs in approximately 25% of medulloblastomas. However, its role in medulloblastoma pathogenesis is not understood. Methods: We have developed a model of WNT/β-catenin pathway-activated medulloblastoma. Pathway activation was induced in a Myc immortalised cerebellar progenitor cell line through stable expression of Wnt1. In vitro and in vivo analysis was undertaken to understand the effect of pathway activation and identify the potential cell of origin. Results: Tumours that histologically resembled classical medulloblastoma formed in vivo using cells overexpressing Wnt1, but not with the control cell line. Wnt1 overexpression inhibited neuronal differentiation in vitro, suggesting WNT/β-catenin pathway activation prevents cells terminally differentiating, maintaining them in a more ‘stem-like’ state. Analysis of cerebellar progenitor cell markers demonstrated the cell line resembled cells from the cerebellar ventricular zone. conclusion: We have developed a cell line with the means of orthotopically modelling WNT/β-catenin pathway-activated medulloblastoma. We provide evidence of the role pathway activation is playing in tumour pathogenesis and suggest medulloblastomas can arise from cells other than granule cell progenitors. This cell line is a valuable resource to further understand the role of pathway activation in tumorigenesis and for investigation of targeted therapies.
first_indexed 2025-11-14T18:18:33Z
format Article
id nottingham-2542
institution University of Nottingham Malaysia Campus
institution_category Local University
last_indexed 2025-11-14T18:18:33Z
publishDate 2012
publisher Cancer Research UK
recordtype eprints
repository_type Digital Repository
spelling nottingham-25422020-05-04T16:33:58Z https://eprints.nottingham.ac.uk/2542/ WNT/β-catenin pathway activation in Myc immortalised cerebellar progenitor cells inhibits neuronal differentiation and generates tumours resembling medulloblastoma Rogers, H.A. Sousa, S. Salto, C. Arenas, E. Coyle, B. Grundy, Richard G. Background: Medulloblastoma is the most common malignant childhood brain tumour. Aberrant activation of the WNT/β-catenin pathway occurs in approximately 25% of medulloblastomas. However, its role in medulloblastoma pathogenesis is not understood. Methods: We have developed a model of WNT/β-catenin pathway-activated medulloblastoma. Pathway activation was induced in a Myc immortalised cerebellar progenitor cell line through stable expression of Wnt1. In vitro and in vivo analysis was undertaken to understand the effect of pathway activation and identify the potential cell of origin. Results: Tumours that histologically resembled classical medulloblastoma formed in vivo using cells overexpressing Wnt1, but not with the control cell line. Wnt1 overexpression inhibited neuronal differentiation in vitro, suggesting WNT/β-catenin pathway activation prevents cells terminally differentiating, maintaining them in a more ‘stem-like’ state. Analysis of cerebellar progenitor cell markers demonstrated the cell line resembled cells from the cerebellar ventricular zone. conclusion: We have developed a cell line with the means of orthotopically modelling WNT/β-catenin pathway-activated medulloblastoma. We provide evidence of the role pathway activation is playing in tumour pathogenesis and suggest medulloblastomas can arise from cells other than granule cell progenitors. This cell line is a valuable resource to further understand the role of pathway activation in tumorigenesis and for investigation of targeted therapies. Cancer Research UK 2012-09-25 Article PeerReviewed Rogers, H.A., Sousa, S., Salto, C., Arenas, E., Coyle, B. and Grundy, Richard G. (2012) WNT/β-catenin pathway activation in Myc immortalised cerebellar progenitor cells inhibits neuronal differentiation and generates tumours resembling medulloblastoma. British Journal of Cancer, 107 . pp. 1144-1152. ISSN 0007-0920 http://www.nature.com/bjc/journal/v107/n7/full/bjc2012377a.html doi:10.1038/bjc.2012.377 doi:10.1038/bjc.2012.377
spellingShingle Rogers, H.A.
Sousa, S.
Salto, C.
Arenas, E.
Coyle, B.
Grundy, Richard G.
WNT/β-catenin pathway activation in Myc immortalised cerebellar progenitor cells inhibits neuronal differentiation and generates tumours resembling medulloblastoma
title WNT/β-catenin pathway activation in Myc immortalised cerebellar progenitor cells inhibits neuronal differentiation and generates tumours resembling medulloblastoma
title_full WNT/β-catenin pathway activation in Myc immortalised cerebellar progenitor cells inhibits neuronal differentiation and generates tumours resembling medulloblastoma
title_fullStr WNT/β-catenin pathway activation in Myc immortalised cerebellar progenitor cells inhibits neuronal differentiation and generates tumours resembling medulloblastoma
title_full_unstemmed WNT/β-catenin pathway activation in Myc immortalised cerebellar progenitor cells inhibits neuronal differentiation and generates tumours resembling medulloblastoma
title_short WNT/β-catenin pathway activation in Myc immortalised cerebellar progenitor cells inhibits neuronal differentiation and generates tumours resembling medulloblastoma
title_sort wnt/β-catenin pathway activation in myc immortalised cerebellar progenitor cells inhibits neuronal differentiation and generates tumours resembling medulloblastoma
url https://eprints.nottingham.ac.uk/2542/
https://eprints.nottingham.ac.uk/2542/
https://eprints.nottingham.ac.uk/2542/