The occurrence of intracranial rhabdoid tumours in mice depends on temporal control of Smarcb1 inactivation

Zhi-Yan Han, Wilfrid Richer, Paul Fréneaux, Céline Chauvin, Carlo Lucchesi, Delphine Guillemot, Camille Grison, Delphine Lequin, Gaelle Pierron, Julien Masliah-Planchon, André Nicolas, Dominique Ranchère-Vince, Pascale Varlet, Stéphanie Puget, Isabelle Janoueix-Lerosey, Olivier Ayrault, Didier Surdez, Olivier Delattre () and Franck Bourdeaut ()
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Zhi-Yan Han: Institut Curie, Paris Sciences et Lettres Research University, InsermU830, Laboratoire de Genetique et Biologie des Cancers
Wilfrid Richer: Institut Curie, Paris Sciences et Lettres Research University, InsermU830, Laboratoire de Genetique et Biologie des Cancers
Paul Fréneaux: Institut Curie, Service d’anatomie pathologique
Céline Chauvin: Institut Curie, Paris Sciences et Lettres Research University, InsermU830, Laboratoire de Genetique et Biologie des Cancers
Carlo Lucchesi: Institut Curie, Paris Sciences et Lettres Research University, InsermU830, Laboratoire de Genetique et Biologie des Cancers
Delphine Guillemot: Institut Bergonie, Institut Curie, Unité de génétique somatique
Camille Grison: Institut Bergonie, Institut Curie, Unité de génétique somatique
Delphine Lequin: Institut Bergonie, Institut Curie, Unité de génétique somatique
Gaelle Pierron: Institut Bergonie, Institut Curie, Unité de génétique somatique
Julien Masliah-Planchon: Institut Bergonie, Institut Curie, Unité de génétique somatique
André Nicolas: Institut Curie, Plateforme de pathologie expérimentale
Dominique Ranchère-Vince: Centre Léon Bérard, Departement de Biopathologie, 28 Promenade Léa et Napoléon Bullukian
Pascale Varlet: Departement de neuropathology, Hopital Sainte-Anne
Stéphanie Puget: Université Paris Descartes
Isabelle Janoueix-Lerosey: Institut Curie, Paris Sciences et Lettres Research University, InsermU830, Laboratoire de Genetique et Biologie des Cancers
Olivier Ayrault: Institut Curie, Paris Sciences et Lettres University Research, CNRS UMR 3306, INSERM U1005, Centre Universitaire d’Orsay
Didier Surdez: Institut Curie, Paris Sciences et Lettres Research University, InsermU830, Laboratoire de Genetique et Biologie des Cancers
Olivier Delattre: Institut Curie, Paris Sciences et Lettres Research University, InsermU830, Laboratoire de Genetique et Biologie des Cancers
Franck Bourdeaut: Institut Curie, Paris Sciences et Lettres Research University, InsermU830, Laboratoire de Genetique et Biologie des Cancers

Nature Communications, 2016, vol. 7, issue 1, 1-11

Abstract: Abstract Rhabdoid tumours (RTs) are highly aggressive tumours of infancy, frequently localized in the central nervous system (CNS) where they are termed atypical teratoid/rhabdoid tumours (AT/RTs) and characterized by bi-allelic inactivation of the SMARCB1 tumour suppressor gene. In this study, by temporal control of tamoxifen injection in Smarcb1flox/flox;Rosa26-CreERT2 mice, we explore the phenotypes associated with Smarcb1 inactivation at different developmental stages. Injection before E6, at birth or at 2 months of age recapitulates previously described phenotypes including embryonic lethality, hepatic toxicity or development of T-cell lymphomas, respectively. Injection between E6 and E10 leads to high penetrance tumours, mainly intra-cranial, with short delays (median: 3 months). These tumours demonstrate anatomical, morphological and gene expression profiles consistent with those of human AT/RTs. Moreover, intra- and inter-species comparisons of tumours reveal that human and mouse RTs can be split into different entities that may underline the variety of RT cells of origin.

Date: 2016
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DOI: 10.1038/ncomms10421

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