Delving deeper into MALT lymphoma biology.
Mucosa-associated lymphoid tissue (MALT) lymphomas can arise in a variety of extranodal sites. Interestingly, at least 3 different, apparently site-specific, chromosomal translocations, all affecting the NF-kappaB pathway, have been implicated in the development and progression of MALT lymphoma. The most common is the translocation t(11;18)(q21;q21), which results in a fusion of the cIAP2 region on chromosome 11q21 with the MALT1 gene on chromosome 18q21 and is present in more than one-third of cases. The frequency of this translocation is site-related: common in the gastrointestinal tract and lung, rare in conjunctiva and orbit, and almost absent in salivary glands, thyroid, liver, and skin. In this issue of the JCI, Hu et al. add to our understanding of the molecular consequences of this translocation, showing that its fusion product, cIAP2-MALT1, may concomitantly contribute to lymphomagenesis both as a tumor suppressor gene and as an oncogene.
Bertoni
Francesco
F
Laboratory of Experimental Oncology and Lymphoma Unit, Oncology Institute of Southern Switzerland, Bellinzona, Switzerland.
Zucca
Emanuele
E
D020169:Caspases; D002880:Chromosomes, Human, Pair 11; D002887:Chromosomes, Human, Pair 18; D004068:Digestive System Physiological Phenomena; D015972:Gene Expression Regulation, Neoplastic; D005787:Gene Frequency; D050939:Gene Fusion; D016147:Genes, Tumor Suppressor; D006801:Humans; D051034:Inhibitor of Apoptosis Proteins / Q000235:genetics*; D018442:Lymphoma, B-Cell, Marginal Zone / Q000235:genetics* / Q000473:pathology; D000074884:Mucosa-Associated Lymphoid Tissue Lymphoma Translocation 1 Protein; D009363:Neoplasm Proteins / Q000235:genetics*; D009857:Oncogenes; D009928:Organ Specificity; D014178:Translocation, Genetic
D051034:Inhibitor of Apoptosis Proteins; D009363:Neoplasm Proteins; D020169:Caspases; C400109:MALT1 protein, human; D000074884:Mucosa-Associated Lymphoid Tissue Lymphoma Translocation 1 Protein