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

Research output: Contribution to journalArticlepeer-review

4 Scopus citations

Abstract

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
Volume132
Issue number12
DOIs
StatePublished - Jun 15 2013
Externally publishedYes

Keywords

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

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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