| http://purl.uniprot.org/citations/36127323 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/36127323 | http://www.w3.org/2000/01/rdf-schema#comment | "Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant brain tumor in infants that is characterized by loss of nuclear expression of SMARCB1 or SMARCA4 proteins. Recent studies show that AT/RTs comprise three molecular subgroups, namely AT/RT-TYR, AT/RT-MYC and AT/RT-SHH. The subgroups show distinct expression patterns of genes involved in ciliogenesis, however, little is known about the functional roles of primary cilia in the biology of AT/RT. Here, we show that primary cilia are present across all AT/RT subgroups with specific enrichment in AT/RT-TYR patient samples. Furthermore, we demonstrate that primary ciliogenesis contributes to AT/RT biology in vitro and in vivo. Specifically, we observed a significant decrease in proliferation and clonogenicity following disruption of primary ciliogenesis in AT/RT cell line models. Additionally, apoptosis was significantly increased via the induction of STAT1 and DR5 signaling, as detected by proteogenomic profiling. In a Drosophila model of SMARCB1 deficiency, concomitant knockdown of several cilia-associated genes resulted in a substantial shift of the lethal phenotype with more than 20% of flies reaching adulthood. We also found significantly extended survival in an orthotopic xenograft mouse model of AT/RT upon disruption of primary ciliogenesis. Taken together, our findings indicate that primary ciliogenesis or its downstream signaling contributes to the aggressiveness of AT/RT and, therefore, may constitute a novel therapeutic target."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.org/dc/terms/identifier | "doi:10.1038/s41419-022-05243-4"xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Fischer U."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Kool M."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Reifenberger G."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Liebau M.C."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Picard D."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Borkhardt A."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Qin N."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Pfister S.M."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Erkek S."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Remke M."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Fruhwald M.C."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Hasselblatt M."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Jeibmann A."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Stuhler K."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Meyer F.D."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Barata J.T."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Kerl K."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Bartl J."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Johann P.D."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Blumel L."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Faria C.C."xsd:string |
| http://purl.uniprot.org/citations/36127323 | http://purl.uniprot.org/core/author | "Pauck D."xsd:string |