Mouse Smarcb1-deficient models recapitulate subtypes of human rhabdoid tumors.

Rhabdoid Tumors (RT) are highly aggressive tumors that are frequently localized in the central nervous system (CNS) where they are termed atypical teratoid and rhabdoid tumors (ATRT). We generated conditional Smarcb1-deficient mouse model leads to CNS Smarcb1-deficient tumors. We used microarrays to compared gene expression profilings of various human and mouse tumors. Our data demonstrate that the Smarcb1-deficient mouse model recapitulates the diversity of human RT. [human mRNA] 20 human MRT ( < 5 years), 30 human ATRT ( < 5 years), 4 human MB SHH. [mouse mRNA] 8 Smarcb1-deficient mouse brain tumors, 15 Smarcb1-deficient T-Cell lymphomas, 3 Mb Shh mouse tumors.