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000275262 1001_ $$aPaassen, Irene$$b0
000275262 245__ $$aAtypical teratoid/rhabdoid tumoroids reveal subgroup-specific drug vulnerabilities.
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000275262 520__ $$aAtypical teratoid/rhabdoid tumors (ATRTs) represent a rare, but aggressive pediatric brain tumor entity. They are genetically defined by alterations in the SWI/SNF chromatin remodeling complex members SMARCB1 or SMARCA4. ATRTs can be further classified in different molecular subgroups based on their epigenetic profiles. Although recent studies suggest that the different subgroups have distinct clinical features, subgroup-specific treatment regimens have not been developed thus far. This is hampered by the lack of pre-clinical in vitro models representative of the different molecular subgroups. Here, we describe the establishment of ATRT tumoroid models from the ATRT-MYC and ATRT-SHH subgroups. We demonstrate that ATRT tumoroids retain subgroup-specific epigenetic and gene expression profiles. High throughput drug screens on our ATRT tumoroids revealed distinct drug sensitivities between and within ATRT-MYC and ATRT-SHH subgroups. Whereas ATRT-MYC universally displayed high sensitivity to multi-targeted tyrosine kinase inhibitors, ATRT-SHH showed a more heterogeneous response with a subset showing high sensitivity to NOTCH inhibitors, which corresponded to high expression of NOTCH receptors. Our ATRT tumoroids represent the first pediatric brain tumor organoid model, providing a representative pre-clinical model which enables the development of subgroup-specific therapies.
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000275262 7001_ $$aWilliams, Justin$$b1
000275262 7001_ $$aRíos Arceo, Carla$$b2
000275262 7001_ $$00000-0002-0684-4613$$aRingnalda, Femke$$b3
000275262 7001_ $$aMercer, Kimberly Shea$$b4
000275262 7001_ $$aBuhl, Juliane L$$b5
000275262 7001_ $$aMoreno, Natalia$$b6
000275262 7001_ $$0P:(DE-He78)32c5110cd42ee8a96b18a3e8909bd0a9$$aFederico, Aniello$$b7$$udkfz
000275262 7001_ $$aFranke, Niels E$$b8
000275262 7001_ $$aKranendonk, Mariette$$b9
000275262 7001_ $$00000-0001-6101-149X$$aUpadhyaya, Santhosh A$$b10
000275262 7001_ $$aKerl, Kornelius$$b11
000275262 7001_ $$avan de Wetering, Marc$$b12
000275262 7001_ $$00000-0002-3077-5582$$aClevers, Hans$$b13
000275262 7001_ $$0P:(DE-He78)4c28e2aade5f44d8eca9dd8e97638ec8$$aKool, Marcel$$b14$$udkfz
000275262 7001_ $$aHoving, Eelco W$$b15
000275262 7001_ $$00000-0002-1740-8139$$aRoussel, Martine F$$b16
000275262 7001_ $$00000-0002-2941-6179$$aDrost, Jarno$$b17
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