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For instance, the translocation t(12;21)(p13;q22), representing the <jats:italic>ETV6<\/jats:italic>\u2010<jats:italic>RUNX1<\/jats:italic> fusion gene (ER), is present in a quarter of BCP\u2010ALL cases. However, around 10% of the cases lack classifying chromosomal abnormalities (B\u2010other). In pediatric ER BCP\u2010ALL, rearrangement mediated by RAG (recombination\u2010activating genes) has been proposed as the predominant driver of oncogenic rearrangement. Herein we analyzed almost 1600 pediatric BCP\u2010ALL samples to determine which subtypes express <jats:italic>RAG<\/jats:italic>. We demonstrate that <jats:italic>RAG1<\/jats:italic> mRNA levels are especially high in the ETV6\u2010RUNX1 (ER) subtype and in a subset of B\u2010other samples. We also define 31 genes that are co\u2010expressed with <jats:italic>RAG1<\/jats:italic> (<jats:italic>RAG1<\/jats:italic>\u2010signature) in the ER subtype, a signature that also identifies this subset of B\u2010other samples. Moreover, this subset also shares leukemia and pro\u2010B gene expression signatures as well as high levels of the <jats:italic>ETV6<\/jats:italic> target genes (<jats:italic>BIRC7<\/jats:italic>, <jats:italic>WBP1L<\/jats:italic>, <jats:italic>CLIC5<\/jats:italic>, <jats:italic>ANGPTL2<\/jats:italic>) with the ER subtype, indicating that these B\u2010other cases are the recently identified ER\u2010like subtype. We validated our results in a cohort where ER\u2010like has been defined, which confirmed expression of the <jats:italic>RAG1<\/jats:italic>\u2010signature in this recently described subtype. Taken together, our results demonstrate that the <jats:italic>RAG1<\/jats:italic>\u2010signature identifies the ER\u2010like subtype. As there are no definitive genetic markers to identify this novel subtype, the <jats:italic>RAG1<\/jats:italic>\u2010signature represents a means to screen for this leukemia in children.<\/jats:p>","DOI":"10.1002\/cam4.3928","type":"journal-article","created":{"date-parts":[[2021,5,14]],"date-time":"2021-05-14T04:06:20Z","timestamp":1620965180000},"page":"3997-4003","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["<i>RAG1<\/i> co\u2010expression signature identifies ETV6\u2010RUNX1\u2010like B\u2010cell precursor acute lymphoblastic leukemia in children"],"prefix":"10.1002","volume":"10","author":[{"given":"Dongfeng","family":"Chen","sequence":"first","affiliation":[{"name":"Institute of Life Sciences Jiangsu University Zhenjiang China"},{"name":"Department of Rheumatology and Inflammation Research Institute of Medicine Sahlgrenska Academy University of Gothenburg Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro","family":"Camponeschi","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Inflammation Research Institute of Medicine Sahlgrenska Academy University of Gothenburg Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica","family":"Nordlund","sequence":"additional","affiliation":[{"name":"Department of Medical Sciences Molecular Medicine and Science for Life Laboratory Uppsala University Uppsala Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yanara","family":"Marincevic\u2010Zuniga","sequence":"additional","affiliation":[{"name":"Department of Medical Sciences Molecular Medicine and Science for Life Laboratory Uppsala University Uppsala Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonas","family":"Abrahamsson","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Institute of Clinical Sciences Sahlgrenska University Hospital Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gudmar","family":"L\u00f6nnerholm","sequence":"additional","affiliation":[{"name":"Department of Women and Children\u2019s Health Uppsala University Uppsala Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda","family":"Fogelstrand","sequence":"additional","affiliation":[{"name":"Department of Clinical Chemistry Sahlgrenska University Hospital Gothenburg Sweden"},{"name":"Department of Clinical Chemistry and Transfusion Medicine University of Gothenburg Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3415-0560","authenticated-orcid":false,"given":"Inga\u2010Lill","family":"M\u00e5rtensson","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Inflammation Research Institute of Medicine Sahlgrenska Academy University of Gothenburg 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