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Rhabdoid sarcomas are rare, lethal pediatric sarcomas characterized by a 22q11 chromosome rearrangement that targets and inactivates the BAF47 (INI1, smarcb1) gene.PMID:28038441 BAF47 has been shown to be inactivated within the vast majority of these tumors via mutations, gene deletions, or both. Having said that, in a minimum of ten of instances, this gene may well be silenced via as however unidentified mechanisms [1]. Heterozygous knockouts of BAF47 yield tumors in 30 of mice, a percentage that has been confirmed by 3 distinctive groups [2]. Homozygous knockouts of BAF47, in comparison, resulted in tumor development in 100 from the mice (80 lymphomaswww.impactjournals.com/oncotargetand 20 Rhabdoid tumors) inside a median time of 1012 weeks [3]. In vitro studies have shown that reexpression of BAF47 in BAF47deficient cell lines yields growth inhibition [4]. Together, these information solidified BAF47’s function as a tumor suppressor that underlies the genesis of Rhabdoid tumors. Though BAF47 silencing is viewed as extremely tumorigenic, this gene has not been fo.