{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Mercher T"],"funding":["NIDDK NIH HHS","NCI NIH HHS"],"pagination":["852-64"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC2662544"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["119(4)"],"pubmed_abstract":["Acute megakaryoblastic leukemia (AMKL) is a form of acute myeloid leukemia (AML) associated with a poor prognosis. The genetics and pathophysiology of AMKL are not well understood. We generated a knockin mouse model of the one twenty-two-megakaryocytic acute leukemia (OTT-MAL) fusion oncogene that results from the t(1;22)(p13;q13) translocation specifically associated with a subtype of pediatric AMKL. We report here that OTT-MAL expression deregulated transcriptional activity of the canonical Notch signaling pathway transcription factor recombination signal binding protein for immunoglobulin kappa J region (RBPJ) and caused abnormal fetal megakaryopoiesis. Furthermore, cooperation between OTT-MAL and an activating mutation of the thrombopoietin receptor myeloproliferative leukemia virus on"],"journal":["The Journal of clinical investigation"],"pubmed_title":["The OTT-MAL fusion oncogene activates RBPJ-mediated transcription and induces acute megakaryoblastic leukemia in a knockin mouse model."],"pmcid":["PMC2662544"],"funding_grant_id":["P01 CA066996","CA66996","DK50654","K08 CA111399","P01 DK050654","U01 CA105423"],"pubmed_authors":["Cornejo MG","Cagnard N","Bernard OA","Moore SA","Williams IR","Jesneck JL","Levine RL","Mercher T","Cullen D","Raffel GD","Baudry-Bluteau D","Bourquin JP","Vainchenker W","Pikman Y","Lee BH","Akashi K","Gilliland DG","Giovannini M","Shigematsu H"],"additional_accession":[]},"is_claimable":false,"name":"The OTT-MAL fusion oncogene activates RBPJ-mediated transcription and induces acute megakaryoblastic leukemia in a knockin mouse model.","description":"Acute megakaryoblastic leukemia (AMKL) is a form of acute myeloid leukemia (AML) associated with a poor prognosis. The genetics and pathophysiology of AMKL are not well understood. We generated a knockin mouse model of the one twenty-two-megakaryocytic acute leukemia (OTT-MAL) fusion oncogene that results from the t(1;22)(p13;q13) translocation specifically associated with a subtype of pediatric AMKL. We report here that OTT-MAL expression deregulated transcriptional activity of the canonical Notch signaling pathway transcription factor recombination signal binding protein for immunoglobulin kappa J region (RBPJ) and caused abnormal fetal megakaryopoiesis. Furthermore, cooperation between OTT-MAL and an activating mutation of the thrombopoietin receptor myeloproliferative leukemia virus on","dates":{"release":"2009-01-01T00:00:00Z","publication":"2009 Apr","modification":"2026-05-03T20:49:08.192Z","creation":"2019-03-27T00:21:29Z"},"accession":"S-EPMC2662544","cross_references":{"pubmed":["19287095"],"doi":["10.1172/JCI35901","10.1172/jci35901"]}}