Establishment and characterization of MRT cell lines from genetically engineered mouse models and the influence of genetic background on their development

Yasumichi Kuwahara, E. Lorena Mora-Blanco, Fatima Banine, Arlin B. Rogers, Christopher Fletcher, Larry S. Sherman, Charles W.M. Roberts, Bernard E. Weissman

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    2 Scopus citations


    Malignant rhabdoid tumors (MRTs) are rare, aggressive cancers occuring in young children primarily through inactivation of the SNF5(INI1, SMARCB1) tumor suppressor gene. We and others have demonstrated that mice heterozygous for a Snf5 null allele develop MRTs with partial penetrance. We have also shown that Snf5+/- mice that lack expression of the pRb family, due to TgT 121 transgene expression, develop MRTs with increased penetrance and decreased latency. Here, we report that altering the genetic background has substantial effects upon MRT development in Snf5+/- and TgT 121;Snf5+/- mice, with a mixed F1 background resulting in increased latency and the appearance of brain tumors. We also report the establishment of the first mouse MRT cell lines that recapitulate many features of their human counterparts. Our studies provide further insight into the genetic influences on MRT development as well as provide valuable new cell culture and genetically engineered mouse models for the study of CNS-MRT etiology.

    Original languageEnglish (US)
    Pages (from-to)2767-2777
    Number of pages11
    JournalInternational Journal of Cancer
    Issue number12
    StatePublished - Jun 15 2013



    • SNF5/INI1
    • SWI/SNF complex
    • mouse models of cancer
    • pediatric cancers

    ASJC Scopus subject areas

    • Oncology
    • Cancer Research

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