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Delving deeper into MALT lymphoma biology
Francesco Bertoni, Emanuele Zucca
Francesco Bertoni, Emanuele Zucca
Published January 4, 2006
Citation Information: J Clin Invest. 2006;116(1):22-26. https://doi.org/10.1172/JCI27476.
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Commentary

Delving deeper into MALT lymphoma biology

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Abstract

Gastric 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-κB 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.

Authors

Francesco Bertoni, Emanuele Zucca

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Figure 1

cIAP2, MALT1, and cIAP2-MALT1 organization.

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cIAP2, MALT1, and cIAP2-MALT1 organization.
Schematic diagram showing th...
Schematic diagram showing the structure of cIAP2 (A), MALT1 (B), and the 2 most commonly observed cIAP2-MALT1 fusion proteins (C), including their known functional domains. The dashed lines show the most frequent breakpoint sites occurring in the t(11;18)(q21;q21) chromosomal translocation. The study by Hu et al. (22) in this issue of the JCI adds to our understanding of the properties of cIAP2, showing BCL10 ubiquitin ligase activity in its COOH-terminal region together with a BCL10 binding site within its NH2-terminal region. RING, really interesting new gene; ID, Ig-like domain; CLD, caspase-like domain.

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ISSN: 0021-9738 (print), 1558-8238 (online)

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