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Title: A BCL6/BCOR/SIRT1 complex triggers neurogenesis and suppresses medulloblastoma by repressing Sonic Hedgehog signaling
Authors: Tiberi, Luca
Bonnefont, Jérôme
van den Ameele, Jelle
Le Bon, Serge-Daniel
Herpoel, Adèle
Bilheu, Angéline
Baron, Beverly W
Vanderhaeghen, Pierre # ×
Issue Date: Dec-2014
Publisher: Cell Press
Series Title: Cancer Cell vol:26 issue:6
Article number: 10.1016/j.ccell.2014.10.021
Abstract: Disrupted differentiation during development can lead to oncogenesis, but the underlying mechanisms remain poorly understood. Here we identify BCL6, a transcriptional repressor and lymphoma oncoprotein, as a pivotal factor required for neurogenesis and tumor suppression of medulloblastoma (MB). BCL6 is necessary for and capable of preventing the development of GNP-derived MB in mice, and can block the growth of human MB cells in vitro. BCL6 neurogenic and oncosuppressor effects rely on direct transcriptional repression of Gli1 and Gli2 effectors of the SHH pathway, through recruitment of BCOR corepressor and SIRT1 deacetylase. Our findings identify the BCL6/BCOR/SIRT1 complex as a potent repressor of the SHH pathway in normal and oncogenic neural development, with direct diagnostic and/or therapeutic relevance for SHH MB.
URI: 
ISSN: 1535-6108
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Non-KU Leuven Association publications
× corresponding author
# (joint) last author

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