Document Detail


Establishment and characterization of MRT cell lines from genetically engineered mouse models and the influence of genetic background on their development.
MedLine Citation:
PMID:  23197309     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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 TgT121 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 TgT121 ;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.
Authors:
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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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2012-12-27
Journal Detail:
Title:  International journal of cancer. Journal international du cancer     Volume:  132     ISSN:  1097-0215     ISO Abbreviation:  Int. J. Cancer     Publication Date:  2013 Jun 
Date Detail:
Created Date:  2013-04-11     Completed Date:  2013-06-12     Revised Date:  2014-06-17    
Medline Journal Info:
Nlm Unique ID:  0042124     Medline TA:  Int J Cancer     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2767-77     Citation Subset:  IM    
Copyright Information:
Copyright © 2012 UICC.
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MeSH Terms
Descriptor/Qualifier:
Animals
Cell Line, Tumor*
Cell Proliferation
Cell Transformation, Neoplastic / genetics
Choroid Plexus Neoplasms / genetics,  pathology
Chromosomal Proteins, Non-Histone / genetics
Disease Models, Animal
Genotype
Humans
Karyotype
Mice
Mice, Transgenic
Phenotype
Rhabdoid Tumor / genetics*,  pathology
Grant Support
ID/Acronym/Agency:
P30 CA016086/CA/NCI NIH HHS; P51 RR000163/RR/NCRR NIH HHS; R01 CA091048/CA/NCI NIH HHS; R01 CA113794/CA/NCI NIH HHS; R01CA091048/CA/NCI NIH HHS; R01CA113794/CA/NCI NIH HHS; RR00163/RR/NCRR NIH HHS; T32 GM007226/GM/NIGMS NIH HHS; U01 CA105423/CA/NCI NIH HHS; U01CA105423/CA/NCI NIH HHS
Chemical
Reg. No./Substance:
0/Chromosomal Proteins, Non-Histone; 0/Smarcb1 protein, mouse
Comments/Corrections

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