{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T06:04:17Z","timestamp":1791180257965,"version":"4.1.0"},"reference-count":25,"publisher":"American Society of Hematology","issue":"18","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2011,11,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Splenic marginal zone lymphoma (SMZL) is one of the few B-cell lymphoma types that remain orphan of molecular lesions in cancer-related genes. Detection of active NF-\u03baB signaling in 14 (58%) of 24 SMZLs prompted the investigation of NF-\u03baB molecular alterations in 101 SMZLs. Mutations and copy number abnormalities of NF-\u03baB genes occurred in 36 (36%) of 101 SMZLs and targeted both canonical (TNFAIP3 and IKBKB) and noncanonical (BIRC3, TRAF3, MAP3K14) NF-\u03baB pathways. Most alterations were mutually exclusive, documenting the existence of multiple independent mechanisms affecting NF-\u03baB in SMZL. BIRC3 inactivation in SMZL recurred because of somatic mutations that disrupted the same RING domain that in extranodal marginal zone lymphoma is removed by the t(11;18) translocation, which points to BIRC3 disruption as a common mechanism across marginal zone B-cell lymphomagenesis. Genetic lesions of NF-\u03baB provide a molecular basis for the pathogenesis of more than 30% of SMZLs and offer a suitable target for NF-\u03baB therapeutic approaches in this lymphoma.<\/jats:p>","DOI":"10.1182\/blood-2011-06-359166","type":"journal-article","created":{"date-parts":[[2011,9,1]],"date-time":"2011-09-01T08:09:34Z","timestamp":1314864574000},"page":"4930-4934","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":171,"title":["Alteration of BIRC3 and multiple other NF-\u03baB pathway genes in splenic marginal zone lymphoma"],"prefix":"10.1182","volume":"118","author":[{"given":"Davide","family":"Rossi","sequence":"first","affiliation":[{"name":"Division of Hematology, Department of Clinical and Experimental Medicine, Amedeo Avogadro University of Eastern Piedmont, Novara, Italy;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Deaglio","sequence":"additional","affiliation":[{"name":"Department of Genetics, Biology and Biochemistry, University of Turin, Turin, Italy;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Dominguez-Sola","sequence":"additional","affiliation":[{"name":"Institute for Cancer Genetics and the Herbert Irving Comprehensive Cancer Center, Columbia University, New York, NY;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Rasi","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Clinical and Experimental Medicine, Amedeo Avogadro University of Eastern Piedmont, Novara, Italy;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tiziana","family":"Vaisitti","sequence":"additional","affiliation":[{"name":"Department of Genetics, Biology and Biochemistry, University of Turin, Turin, Italy;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudio","family":"Agostinelli","sequence":"additional","affiliation":[{"name":"Haematopathology, Department L. & A. 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