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

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  • Zhi-Yan Han

    (Institut Curie, Paris Sciences et Lettres Research University, InsermU830, Laboratoire de Genetique et Biologie des Cancers
    SiRIC- Institut Curie, Laboratoire de Recherche Translationnelle en Oncologie Pédiatrique)

  • Wilfrid Richer

    (Institut Curie, Paris Sciences et Lettres Research University, InsermU830, Laboratoire de Genetique et Biologie des Cancers
    SiRIC- Institut Curie, Laboratoire de Recherche Translationnelle en Oncologie Pédiatrique)

  • 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
    SiRIC- Institut Curie, Laboratoire de Recherche Translationnelle en Oncologie Pédiatrique)

  • 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
    Service de neurochirurgie pédiatrique, Hopital Necker)

  • 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
    Institut Bergonie, Institut Curie, Unité de génétique somatique)

  • Franck Bourdeaut

    (Institut Curie, Paris Sciences et Lettres Research University, InsermU830, Laboratoire de Genetique et Biologie des Cancers
    SiRIC- Institut Curie, Laboratoire de Recherche Translationnelle en Oncologie Pédiatrique
    Institut Curie)

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.

Suggested Citation

  • Zhi-Yan Han & Wilfrid Richer & Paul Fréneaux & Céline Chauvin & Carlo Lucchesi & Delphine Guillemot & Camille Grison & Delphine Lequin & Gaelle Pierron & Julien Masliah-Planchon & André Nicolas & Domi, 2016. "The occurrence of intracranial rhabdoid tumours in mice depends on temporal control of Smarcb1 inactivation," Nature Communications, Nature, vol. 7(1), pages 1-11, April.
    • Handle: RePEc:nat:natcom:v:7:y:2016:i:1:d:10.1038_ncomms10421
    DOI: 10.1038/ncomms10421
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    Cited by:

    1. Monika Graf & Marta Interlandi & Natalia Moreno & Dörthe Holdhof & Carolin Göbel & Viktoria Melcher & Julius Mertins & Thomas K. Albert & Dennis Kastrati & Amelie Alfert & Till Holsten & Flavia de Far, 2022. "Single-cell transcriptomics identifies potential cells of origin of MYC rhabdoid tumors," Nature Communications, Nature, vol. 13(1), pages 1-19, December.
    2. María-Jesús Lobón-Iglesias & Mamy Andrianteranagna & Zhi-Yan Han & Céline Chauvin & Julien Masliah-Planchon & Valeria Manriquez & Arnault Tauziede-Espariat & Sandrina Turczynski & Rachida Bouarich-Bou, 2023. "Imaging and multi-omics datasets converge to define different neural progenitor origins for ATRT-SHH subgroups," Nature Communications, Nature, vol. 14(1), pages 1-21, December.
    3. Ulrik Kristoffer Stoltze & Jon Foss-Skiftesvik & Thomas van Overeem Hansen & Simon Rasmussen & Konrad J. Karczewski & Karin A. W. Wadt & Kjeld Schmiegelow, 2024. "The evolutionary impact of childhood cancer on the human gene pool," Nature Communications, Nature, vol. 15(1), pages 1-15, December.

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