{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T20:07:49Z","timestamp":1792786069905,"version":"4.5.0"},"reference-count":34,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T00:00:00Z","timestamp":1777852800000},"content-version":"vor","delay-in-days":1,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"RCD Foundation Scientific Fellowship","id":[{"id":"https:\/\/ror.org\/036p67w50","id-type":"ROR","asserted-by":"crossref"}]},{"DOI":"10.13039\/100001512","name":"NBTS","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100001512","id-type":"DOI","asserted-by":"publisher"}]},{"name":"St. Baldrick\u2019s Foundation Career Development","id":[{"id":"https:\/\/ror.org\/02xjhr934","id-type":"ROR","asserted-by":"crossref"}]},{"name":"Matthew Larson Brain Tumor Research"},{"name":"V Scholar Translational Grant"},{"name":"St. Jude Children\u2019s Research Hospital Research Collaborative on Transcription Regulation in Pediatric Cancer","id":[{"id":"https:\/\/ror.org\/02r3e0967","id-type":"ROR","asserted-by":"crossref"}]},{"name":"Alex\u2019s Lemonade Stand Foundation \u201cA\u201d Award","award":["R01NS128184"],"award-info":[{"award-number":["R01NS128184"]}],"id":[{"id":"https:\/\/ror.org\/038ja4880","id-type":"ROR","asserted-by":"crossref"}]},{"name":"Alex\u2019s Lemonade Stand Foundation \u201cA\u201d Award","award":["R01CA280203"],"award-info":[{"award-number":["R01CA280203"]}],"id":[{"id":"https:\/\/ror.org\/038ja4880","id-type":"ROR","asserted-by":"crossref"}]},{"name":"Alex\u2019s Lemonade Stand Foundation \u201cA\u201d Award","award":["R01CA284455"],"award-info":[{"award-number":["R01CA284455"]}],"id":[{"id":"https:\/\/ror.org\/038ja4880","id-type":"ROR","asserted-by":"crossref"}]},{"name":"Alex\u2019s Lemonade Stand Foundation \u201cA\u201d Award","award":["U01CA281823"],"award-info":[{"award-number":["U01CA281823"]}],"id":[{"id":"https:\/\/ror.org\/038ja4880","id-type":"ROR","asserted-by":"crossref"}]},{"name":"Alex\u2019s Lemonade Stand Foundation \u201cA\u201d Award","award":["U01CA294103"],"award-info":[{"award-number":["U01CA294103"]}],"id":[{"id":"https:\/\/ror.org\/038ja4880","id-type":"ROR","asserted-by":"crossref"}]},{"name":"Alex\u2019s Lemonade Stand Foundation \u201cA\u201d Award","award":["CA220510"],"award-info":[{"award-number":["CA220510"]}],"id":[{"id":"https:\/\/ror.org\/038ja4880","id-type":"ROR","asserted-by":"crossref"}]},{"name":"DOD-IMPACT","award":["CA220247"],"award-info":[{"award-number":["CA220247"]}]},{"name":"St Jude Pediatric Dependency Map Project"},{"name":"Hartwell Center for Biotechnology"},{"name":"Center for Advanced Genome Engineering"},{"name":"National Cancer Institutes","award":["P30 CA021765"],"award-info":[{"award-number":["P30 CA021765"]}]},{"name":"CERN Foundation"},{"DOI":"10.13039\/501100016057","name":"Robert Connor Dawes Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100016057","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,10,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Ependymoma (EPN) is the third most common pediatric brain tumor with no targeted therapies available to patients. In supratentorial ependymoma, the most frequent driver alteration is a gene fusion between ZFTA and RELA (denoted ZR), which leads to constitutive localization of ZR in the nucleus. Because ZR is not currently druggable, we tested whether ZR expression leads to aberrant protein interactions that could represent therapeutic vulnerabilities.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Using CRISPR-Cas9 pooled screening, we identified many novel druggable ZR-interacting proteins including XPO1, CARM1, SMARCA4, and CDK1. We focused on the nuclear export protein (XPO1), given the ability of most XPO1 inhibitors (ie, selinexor) to cross the blood-brain barrier, FDA approval, and have documented safety profiles in children.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>We found that specific nuclear ZR levels are needed for cell proliferation and are regulated by XPO1. Increased ZR accumulation in the nucleus does not increase oncogenic gene expression but drives tumor cells out of the cell cycle, as compared to a defective ZR DNA-binding mutant. Treatment of ZR-driven patient-derived mouse models with selinexor impairs cell growth and extends survival of animals in vivo. The combination of selinexor treatment with gemcitabine and ribociclib (used in a clinical trial for relapsed EPN at St. Jude Children\u2019s Research Hospital [SJDAWN]) further extends mouse survival.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Our findings demonstrate that ZR-interacting proteins constitute therapeutic leads and that XPO1 is critical for titrating \u201cgoldilocks\u201d levels of ZR nuclear expression. We identify a novel combination therapy of selinexor, gemcitabine, and ribociclib that may be immediately translated into clinical trials for EPN patients that are currently without targeted treatments.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/neuonc\/noag100","type":"journal-article","created":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T12:10:39Z","timestamp":1777637439000},"page":"2472-2484","source":"Crossref","is-referenced-by-count":1,"title":["Nuclear export as a therapeutic vulnerability in ZFTA-RELA ependymoma"],"prefix":"10.1093","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4714-8390","authenticated-orcid":false,"given":"Siri M","family":"Ippagunta","sequence":"first","affiliation":[{"name":"Department of Developmental Neurobiology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Center of Excellence in Neuro-Oncology Sciences, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-1921-1193","authenticated-orcid":false,"given":"Amir","family":"Arabzade","sequence":"additional","affiliation":[{"name":"Department of Developmental Neurobiology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Center of Excellence in Neuro-Oncology Sciences, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Department of Chemical Engineering, Rice University , Houston, Texas,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2033-0758","authenticated-orcid":false,"given":"Srinidhi","family":"Varadharajan","sequence":"additional","affiliation":[{"name":"Department of Developmental Neurobiology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Center of Excellence in Neuro-Oncology Sciences, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-0764-6220","authenticated-orcid":false,"given":"Erik","family":"Emanus","sequence":"additional","affiliation":[{"name":"Department of Developmental Neurobiology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Center of Excellence in Neuro-Oncology Sciences, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-8218-0296","authenticated-orcid":false,"given":"Amanda","family":"Bland","sequence":"additional","affiliation":[{"name":"Department of Developmental Neurobiology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Center of Excellence in Neuro-Oncology Sciences, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7438-2107","authenticated-orcid":false,"given":"Alisha","family":"Kardian","sequence":"additional","affiliation":[{"name":"Department of Developmental Neurobiology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Center of Excellence in Neuro-Oncology Sciences, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Baylor College of Medicine Cancer and Cell Biology Program, , Houston, Texas,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-2451-5999","authenticated-orcid":false,"given":"Nicholas","family":"Laboe","sequence":"additional","affiliation":[{"name":"Department of Developmental Neurobiology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Center of Excellence in Neuro-Oncology Sciences, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2169-0808","authenticated-orcid":false,"given":"Hua","family":"Sun","sequence":"additional","affiliation":[{"name":"Department of Developmental Neurobiology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Center of Excellence in Neuro-Oncology Sciences, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3385-7353","authenticated-orcid":false,"given":"Tuyu","family":"Zheng","sequence":"additional","affiliation":[{"name":"Department of Developmental Neurobiology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Center of Excellence in Neuro-Oncology Sciences, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amelia","family":"Hancock","sequence":"additional","affiliation":[{"name":"Department of Developmental Neurobiology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Center of Excellence in Neuro-Oncology Sciences, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-4694-2823","authenticated-orcid":false,"given":"Mark","family":"Zhai","sequence":"additional","affiliation":[{"name":"Department of Developmental Neurobiology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Center of Excellence in Neuro-Oncology Sciences, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emma","family":"Caesar","sequence":"additional","affiliation":[{"name":"Department of Developmental Neurobiology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Center of Excellence in Neuro-Oncology Sciences, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maxwell","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Developmental Neurobiology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Center of Excellence in Neuro-Oncology Sciences, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-6050-1593","authenticated-orcid":false,"given":"Wilda","family":"Orisme","sequence":"additional","affiliation":[{"name":"Department of Developmental Neurobiology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3794-3518","authenticated-orcid":false,"given":"Melissa","family":"Johnson","sequence":"additional","affiliation":[{"name":"Center for In Vivo Imaging and Therapeutics, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brittany","family":"Rodgers","sequence":"additional","affiliation":[{"name":"Center for In Vivo Imaging and Therapeutics, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9659-8291","authenticated-orcid":false,"given":"Gregory H","family":"Turner","sequence":"additional","affiliation":[{"name":"Center for In Vivo Imaging and Therapeutics, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4110-3586","authenticated-orcid":false,"given":"Jeffrey","family":"Steinberg","sequence":"additional","affiliation":[{"name":"Center for In Vivo Imaging and Therapeutics, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Burgess","family":"Freeman","sequence":"additional","affiliation":[{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1740-8139","authenticated-orcid":false,"given":"Martine F","family":"Roussel","sequence":"additional","affiliation":[{"name":"Department of Tumor Cell Biology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6266-2910","authenticated-orcid":false,"given":"Anang","family":"Shelat","sequence":"additional","affiliation":[{"name":"Department of Chemical Biology and Therapeutics, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard W","family":"Kriwacki","sequence":"additional","affiliation":[{"name":"St Jude Children's Research Hospital Department of Structural Biology, , Memphis, Tennessee,","place":["USA"]},{"name":"Cancer Biology Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0412-6255","authenticated-orcid":false,"given":"Thomas E","family":"Merchant","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6335-1081","authenticated-orcid":false,"given":"Benjamin","family":"Deneen","sequence":"additional","affiliation":[{"name":"Center for Cancer Neuroscience, Baylor College of Medicine , Houston, Texas,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7009-3466","authenticated-orcid":false,"given":"Michael D","family":"Taylor","sequence":"additional","affiliation":[{"name":"Texas Children\u2019s Hospital, Baylor College of Medicine , Houston, Texas,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6584-3795","authenticated-orcid":false,"given":"Sameer","family":"Agnihotri","sequence":"additional","affiliation":[{"name":"Department of Neurological Surgery, University of Pittsburgh , Pittsburgh, Pennsylvania,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3793-585X","authenticated-orcid":false,"given":"Shondra","family":"Pruett-Miller","sequence":"additional","affiliation":[{"name":"Center for Advanced Genome Engineering, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9924-2226","authenticated-orcid":false,"given":"Jacob A","family":"Steele","sequence":"additional","affiliation":[{"name":"Center for Advanced Genome Engineering, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3874-3101","authenticated-orcid":false,"given":"Anoop Murthy","family":"Kavirayani","sequence":"additional","affiliation":[{"name":"Department of Pathology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1239-7757","authenticated-orcid":false,"given":"David W","family":"Ellison","sequence":"additional","affiliation":[{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Department of Pathology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1005-2413","authenticated-orcid":false,"given":"Arzu","family":"Onar-Thomas","sequence":"additional","affiliation":[{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Department of Biostatistics, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7441-9486","authenticated-orcid":false,"given":"Giles W","family":"Robinson","sequence":"additional","affiliation":[{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"St Jude Children's Research Hospital Department of Oncology, , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9620-4742","authenticated-orcid":false,"given":"Stephen C","family":"Mack","sequence":"additional","affiliation":[{"name":"Department of Developmental Neurobiology, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"Center of Excellence in Neuro-Oncology Sciences, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kelsey C","family":"Bertrand","sequence":"additional","affiliation":[{"name":"Neurobiology and Brain Tumor Program, St. Jude Children\u2019s Research Hospital , Memphis, Tennessee,","place":["USA"]},{"name":"St Jude Children's Research Hospital Department of Oncology, , Memphis, Tennessee,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2026,5,3]]},"reference":[{"key":"2026100502521391800_noag100-B1","doi-asserted-by":"publisher","first-page":"208","DOI":"10.1038\/s41568-021-00433-2","article-title":"The biology of ependymomas and emerging novel therapies","volume":"22","author":"Saleh","year":"2022","journal-title":"Nat Rev Cancer."},{"key":"2026100502521391800_noag100-B2","doi-asserted-by":"publisher","first-page":"728","DOI":"10.1016\/j.ccell.2015.04.002","article-title":"Molecular classification of ependymal tumors across all CNS compartments, histopathological grades, and age groups","volume":"27","author":"Pajtler","year":"2015","journal-title":"Cancer Cell"},{"key":"2026100502521391800_noag100-B3","doi-asserted-by":"publisher","first-page":"211","DOI":"10.1007\/s00401-018-1877-0","article-title":"Molecular heterogeneity and CXorf67 alterations in posterior fossa group A (PFA) ependymomas","volume":"136","author":"Pajtler","year":"2018","journal-title":"Acta Neuropathol."},{"key":"2026100502521391800_noag100-B4","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1038\/nature25169","article-title":"Therapeutic targeting of ependymoma as informed by oncogenic enhancer profiling","volume":"553","author":"Mack","year":"2018","journal-title":"Nature."},{"key":"2026100502521391800_noag100-B5","doi-asserted-by":"publisher","first-page":"445","DOI":"10.1038\/nature13108","article-title":"Epigenomic alterations define lethal CIMP-positive ependymomas of infancy","volume":"506","author":"Mack","year":"2014","journal-title":"Nature."},{"key":"2026100502521391800_noag100-B6","doi-asserted-by":"publisher","first-page":"143","DOI":"10.1016\/j.ccr.2011.07.007","article-title":"Delineation of two clinically and molecularly distinct subgroups of posterior fossa ependymoma","volume":"20","author":"Witt","year":"2011","journal-title":"Cancer Cell."},{"key":"2026100502521391800_noag100-B7","doi-asserted-by":"publisher","first-page":"eabc0497","DOI":"10.1126\/scitranslmed.abc0497","article-title":"Targeting integrated epigenetic and metabolic pathways in lethal childhood PFA ependymomas","volume":"13","author":"Panwalkar","year":"2021","journal-title":"Sci Transl Med."},{"key":"2026100502521391800_noag100-B8","doi-asserted-by":"publisher","first-page":"108023","DOI":"10.1016\/j.celrep.2020.108023","article-title":"Single-cell RNA sequencing of childhood ependymoma reveals neoplastic cell subpopulations that impact molecular classification and etiology","volume":"32","author":"Gillen","year":"2020","journal-title":"Cell Rep."},{"key":"2026100502521391800_noag100-B9","doi-asserted-by":"publisher","first-page":"4926","DOI":"10.1016\/j.cell.2024.06.023","article-title":"TULIPs decorate the three-dimensional genome of PFA ependymoma","volume":"187","author":"Johnston","year":"2024","journal-title":"Cell."},{"key":"2026100502521391800_noag100-B10","doi-asserted-by":"publisher","first-page":"340","DOI":"10.1016\/j.ccell.2025.11.002","article-title":"Advancing CNS tumor diagnostics with expanded DNA methylation-based classification","volume":"44","author":"Sill","year":"2026","journal-title":"Cancer Cell."},{"key":"2026100502521391800_noag100-B11","doi-asserted-by":"publisher","first-page":"451","DOI":"10.1038\/nature13109","article-title":"C11orf95-RELA fusions drive oncogenic NF-kappaB signalling in ependymoma","volume":"506","author":"Parker","year":"2014","journal-title":"Nature."},{"key":"2026100502521391800_noag100-B12","doi-asserted-by":"publisher","first-page":"2200","DOI":"10.1158\/2159-8290.Cd-20-1066","article-title":"ZFTA-RELA dictates oncogenic transcriptional programs to drive aggressive supratentorial ependymoma","volume":"11","author":"Arabzade","year":"2021","journal-title":"Cancer Discov."},{"key":"2026100502521391800_noag100-B13","doi-asserted-by":"publisher","first-page":"2230","DOI":"10.1158\/2159-8290.Cd-20-0963","article-title":"Cross-species genomics reveals oncogenic dependencies in ZFTA\/C11orf95 Fusion-Positive supratentorial ependymomas","volume":"11","author":"Zheng","year":"2021","journal-title":"Cancer Discov."},{"key":"2026100502521391800_noag100-B14","doi-asserted-by":"publisher","first-page":"3787","DOI":"10.1016\/j.celrep.2018.04.099","article-title":"A De novo mouse model of c11orf95-rela fusion-driven ependymoma identifies driver functions in addition to NF-kappaB","volume":"23","author":"Ozawa","year":"2018","journal-title":"Cell Rep."},{"key":"2026100502521391800_noag100-B15","doi-asserted-by":"publisher","first-page":"1496","DOI":"10.1038\/s41556-025-01745-3","article-title":"Synthetic ZFTA fusions pinpoint disordered protein domain acquisition as a mechanism of brain tumorigenesis","volume":"27","author":"Arabzade","year":"2025","journal-title":"Nat Cell Biol."},{"key":"2026100502521391800_noag100-B16","doi-asserted-by":"publisher","first-page":"2216","DOI":"10.1158\/2159-8290.Cd-20-1052","article-title":"ZFTA translocations constitute ependymoma chromatin remodeling and transcription factors","volume":"11","author":"Kupp","year":"2021","journal-title":"Cancer Discov."},{"key":"2026100502521391800_noag100-B17","doi-asserted-by":"publisher","first-page":"554","DOI":"10.1186\/s13059-014-0554-4","article-title":"MAGeCK enables robust identification of essential genes from genome-scale CRISPR\/Cas9 knockout screens","volume":"15","author":"Li","year":"2014","journal-title":"Genome Biol."},{"key":"2026100502521391800_noag100-B18","doi-asserted-by":"publisher","first-page":"323","DOI":"10.1016\/j.ccr.2005.09.001","article-title":"Radial glia cells are candidate stem cells of ependymoma","volume":"8","author":"Taylor","year":"2005","journal-title":"Cancer Cell."},{"key":"2026100502521391800_noag100-B19","doi-asserted-by":"publisher","first-page":"1175","DOI":"10.1038\/ncb1638","article-title":"Inhibition of Crm1-p53 interaction and nuclear export of p53 by poly(ADP-ribosyl)ation","volume":"9","author":"Kanai","year":"2007","journal-title":"Nat Cell Biol."},{"key":"2026100502521391800_noag100-B20","doi-asserted-by":"publisher","first-page":"472","DOI":"10.1158\/0008-5472.Can-21-2964","article-title":"Inhibition of XPO1 sensitizes small cell lung cancer to first- and second-line chemotherapy","volume":"82","author":"Quintanal-Villalonga","year":"2022","journal-title":"Cancer Res."},{"key":"2026100502521391800_noag100-B21","doi-asserted-by":"publisher","first-page":"4166","DOI":"10.1182\/blood-2012-08-447581","article-title":"Prognostic impact and targeting of CRM1 in acute myeloid leukemia","volume":"121","author":"Kojima","year":"2013","journal-title":"Blood."},{"key":"2026100502521391800_noag100-B22","doi-asserted-by":"publisher","first-page":"1587","DOI":"10.1158\/1078-0432.Ccr-24-2754","article-title":"Phase I trial of selinexor in pediatric recurrent\/refractory solid and CNS tumors (ADVL1414): a children\u2019s oncology group phase I consortium trial","volume":"31","author":"Green","year":"2025","journal-title":"Clin Cancer Res."},{"key":"2026100502521391800_noag100-B23","doi-asserted-by":"publisher","first-page":"697","DOI":"10.1093\/neuonc\/nou303","article-title":"Preclinical antitumor efficacy of selective exportin 1 inhibitors in glioblastoma","volume":"17","author":"Green","year":"2015","journal-title":"Neuro Oncol."},{"key":"2026100502521391800_noag100-B24","doi-asserted-by":"publisher","first-page":"wuaf018","DOI":"10.1093\/neuped\/wuaf018","article-title":"Targeting the p53 pathway to treat atypical teratoid rhabdoid tumors","volume":"2","author":"Refaat","year":"2026","journal-title":"Neurooncol Pediatr."},{"key":"2026100502521391800_noag100-B25","doi-asserted-by":"publisher","first-page":"1338","DOI":"10.1158\/1078-0432.Ccr-19-1728","article-title":"Preclinical assessment with clinical validation of selinexor with gemcitabine and Nab-Paclitaxel for the treatment of pancreatic ductal adenocarcinoma","volume":"26","author":"Azmi","year":"2020","journal-title":"Clin Cancer Res."},{"key":"2026100502521391800_noag100-B26","doi-asserted-by":"publisher","first-page":"eadf7006","DOI":"10.1126\/scitranslmed.adf7006","article-title":"Exportin 1 inhibition prevents neuroendocrine transformation through SOX2 down-regulation in lung and prostate cancers","volume":"15","author":"Quintanal-Villalonga","year":"2023","journal-title":"Sci Transl Med."},{"key":"2026100502521391800_noag100-B27","doi-asserted-by":"publisher","first-page":"61","DOI":"10.1186\/s13045-020-00903-4","article-title":"XPO1-dependent nuclear export as a target for cancer therapy","volume":"13","author":"Azizian","year":"2020","journal-title":"J Hematol Oncol."},{"key":"2026100502521391800_noag100-B28","doi-asserted-by":"publisher","first-page":"2139","DOI":"10.1007\/s00432-022-04247-z","article-title":"The efficacy of selinexor (KPT-330), an XPO1 inhibitor, on non-hematologic cancers: a comprehensive review","volume":"149","author":"Landes","year":"2023","journal-title":"J Cancer Res Clin Oncol."},{"key":"2026100502521391800_noag100-B29","doi-asserted-by":"publisher","first-page":"e1513","DOI":"10.1002\/ctm2.1513","article-title":"Molecular analysis of XPO1 inhibitor and gemcitabine-nab-paclitaxel combination in KPC pancreatic cancer mouse model","volume":"13","author":"Uddin","year":"2023","journal-title":"Clin Transl Med."},{"key":"2026100502521391800_noag100-B30","doi-asserted-by":"publisher","first-page":"580","DOI":"10.1093\/neuonc\/nop056","article-title":"A clinically relevant orthotopic xenograft model of ependymoma that maintains the genomic signature of the primary tumor and preserves cancer stem cells in vivo","volume":"12","author":"Yu","year":"2010","journal-title":"Neuro Oncol."},{"key":"2026100502521391800_noag100-B31","doi-asserted-by":"publisher","first-page":"1306","DOI":"10.1158\/1535-7163.Mct-21-0598","article-title":"Combination of ribociclib and gemcitabine for the treatment of medulloblastoma","volume":"21","author":"Pribnow","year":"2022","journal-title":"Mol Cancer Ther."},{"key":"2026100502521391800_noag100-B32","doi-asserted-by":"publisher","first-page":"1209","DOI":"10.1038\/nmeth.2689","article-title":"DGIdb: mining the druggable genome","volume":"10","author":"Griffith","year":"2013","journal-title":"Nat Methods."},{"key":"2026100502521391800_noag100-B33","doi-asserted-by":"publisher","first-page":"100895","DOI":"10.1016\/j.neo.2023.100895","article-title":"Why haven\u2019t we solved intracranial pediatric ependymoma? Current questions and barriers to treatment advances","volume":"39","author":"Hwang","year":"2023","journal-title":"Neoplasia."},{"key":"2026100502521391800_noag100-B34","doi-asserted-by":"publisher","first-page":"1262","DOI":"10.1016\/j.ccell.2021.07.003","article-title":"TRIM8 modulates the EWS\/FLI oncoprotein to promote survival in ewing sarcoma","volume":"39","author":"Seong","year":"2021","journal-title":"Cancer Cell."}],"container-title":["Neuro-Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/neuro-oncology\/advance-article-pdf\/doi\/10.1093\/neuonc\/noag100\/68207609\/noag100.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/neuro-oncology\/article-pdf\/28\/10\/2472\/68207609\/noag100.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/neuro-oncology\/article-pdf\/28\/10\/2472\/68207609\/noag100.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T06:52:19Z","timestamp":1791183139000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/neuro-oncology\/article\/28\/10\/2472\/8666937"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5,3]]},"references-count":34,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2026,5,3]]},"published-print":{"date-parts":[[2026,10,1]]}},"URL":"https:\/\/doi.org\/10.1093\/neuonc\/noag100","relation":{},"ISSN":["1522-8517","1523-5866"],"issn-type":[{"value":"1522-8517","type":"print"},{"value":"1523-5866","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2026,10]]},"published":{"date-parts":[[2026,5,3]]}}}