ArticleCancer research2012
OTX2 represses myogenic and neuronal differentiation in medulloblastoma cells.
Article in Cancer research, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers, 1 of them a synthesis that pooled it.
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Who cites it
28 citing papers in PubMed, 1 synthesis or guideline pooled it, 43 citations in OpenAlex.
- Of travelling homeoproteins and medulloblastomas.Oncogene · 2025Pooled it
- Overexpression of OTX2 in human neural cells links depression risk genes.Translational psychiatry · 2025Article
- Arrested development: the dysfunctional life history of medulloblastoma.Genes & development · 2025Review
- Dissecting super-enhancer driven transcriptional dependencies reveals novel therapeutic strategies and targets for group 3 subtype medulloblastoma.Journal of experimental & clinical cancer research : CR · 2022Article
- The landscape of chromatin accessibility in skeletal muscle during embryonic development in pigs.Journal of animal science and biotechnology · 2021Article
- OTX2 regulates CFTR expression during endoderm differentiation and occupies 3' cis-regulatory elements.Developmental dynamics : an official publication of the American Association of Anatomists · 2021Article
- Impact of Reck expression and promoter activity in neuronal in vitro differentiation.Molecular biology reports · 2021Article
- Otx2 promotes granule cell precursor proliferation and Shh-dependent medulloblastoma maintenance in vivo.Oncogenesis · 2018Article
- Characterization of a novel OTX2-driven stem cell program in Group 3 and Group 4 medulloblastoma.Molecular oncology · 2018Article
- Characterization of brain tumor initiating cells isolated from an animal model of CNS primitive neuroectodermal tumors.Oncotarget · 2018Article
- MRI features as a helpful tool to predict the molecular subgroups of medulloblastoma: state of the art.Therapeutic advances in neurological disorders · 2018Review
- OTX2 Activity at Distal Regulatory Elements Shapes the Chromatin Landscape of Group 3 Medulloblastoma.Cancer discovery · 2017Article
- OTX2 expression contributes to proliferation and progression in Myc-amplified medulloblastoma.American journal of cancer research · 2017Review
- Heterozygous mutation inMolecular vision · 2017Article
- A Proteogenomic Approach to Understanding MYC Function in Metastatic Medulloblastoma Tumors.International journal of molecular sciences · 2016Review
- Genetic and molecular alterations across medulloblastoma subgroups.Journal of molecular medicine (Berlin, Germany) · 2015Review
- Brain Penetration and Efficacy of Different Mebendazole Polymorphs in a Mouse Brain Tumor Model.Clinical cancer research : an official journal of the American Association for Cancer Research · 2015Article
- MiR-206, a Cerebellum Enriched miRNA Is Downregulated in All Medulloblastoma Subgroups and Its Overexpression Is Necessary for Growth Inhibition of Medulloblastoma Cells.Journal of molecular neuroscience : MN · 2015Article
- Effective treatment of diverse medulloblastoma models with mebendazole and its impact on tumor angiogenesis.Neuro-oncology · 2015Article
- Clostridium novyi-NT can cause regression of orthotopically implanted glioblastomas in rats.Oncotarget · 2015Article
Corrections and comments
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Authors and funding
5 authors at 3 institutions in 1 country.
Funding
Abstract
The brain development transcription factor OTX2 is overexpressed and/or genomically amplified in most medulloblastomas, but the mechanistic basis for its contributions in this setting are not understood. In this study, we identified OTX2 as a transcriptional repressor and a gatekeeper of myogenic and neuronal differentiation in medulloblastoma cells. OTX2 binds to the MyoD1 core enhancer through its homeobox domain, and the remarkable repressor activity exhibited by the homeobox domain renders OTX2 transcriptionally repressive. RNA interference-mediated attenuation of OTX2 expression triggered myogenic and neuronal differentiation in vitro and prolonged the survival in an orthotopic medulloblastoma mouse model. Conversely, inducing myogenic conversion of medulloblastoma cells led to the loss of OTX2 expression. In medullomyoblastoma, a medulloblastoma subtype containing muscle elements, myogenic cells share cytogenetic signatures with the primitive tumor cells and OTX2 expression was lost in the differentiated myogenic cells. Thus, OTX2 functions via its homeobox domain as a suppressor of differentiation, and the loss of OTX2 expression is linked to the myogenesis in medullomyoblastoma. Together, our findings illustrate the origin of muscle cells in medullomyoblastomas and the oncogenic mechanism of OTX2 as a repressor of diverse differentiating potential.
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Registered trials
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