PMID- 10319863 OWN - NLM STAT- MEDLINE DCOM- 19990525 LR - 20171116 IS - 1061-4036 (Print) IS - 1061-4036 (Linking) VI - 22 IP - 1 DP - 1999 May TI - Inactivating mutations and overexpression of BCL10, a caspase recruitment domain-containing gene, in MALT lymphoma with t(1;14)(p22;q32). PG - 63-8 AB - Mucosa-associated lymphoid tissue (MALT) lymphomas most frequently involve the gastrointestinal tract and are the most common subset of extranodal non-Hodgkin lymphoma (NHL). Here we describe overexpression of BCL10, a novel apoptotic signalling gene that encodes an amino-terminal caspase recruitment domain (CARD), in MALT lymphomas due to the recurrent t(1;14)(p22;q32). BCL10 cDNAs from t(1;14)-positive MALT tumours contained a variety of mutations, most resulting in truncations either in or carboxy terminal to the CARD. Wild-type BCL10 activated NF-kappaB but induced apoptosis of MCF7 and 293 cells. CARD-truncation mutants were unable to induce cell death or activate NF-kappaB, whereas mutants with C-terminal truncations retained NF-kappaB activation but did not induce apoptosis. Mutant BCL10 overexpression might have a twofold lymphomagenic effect: loss of BCL10 pro-apoptosis may confer a survival advantage to MALT B-cells, and constitutive NF-kappaB activation may provide both anti-apoptotic and proliferative signals mediated via its transcriptional targets. FAU - Zhang, Q AU - Zhang Q AD - Department of Pathology and Laboratory Medicine, St. Jude Children's Research Hospital, Memphis, Tennessee 38105, USA. FAU - Siebert, R AU - Siebert R FAU - Yan, M AU - Yan M FAU - Hinzmann, B AU - Hinzmann B FAU - Cui, X AU - Cui X FAU - Xue, L AU - Xue L FAU - Rakestraw, K M AU - Rakestraw KM FAU - Naeve, C W AU - Naeve CW FAU - Beckmann, G AU - Beckmann G FAU - Weisenburger, D D AU - Weisenburger DD FAU - Sanger, W G AU - Sanger WG FAU - Nowotny, H AU - Nowotny H FAU - Vesely, M AU - Vesely M FAU - Callet-Bauchu, E AU - Callet-Bauchu E FAU - Salles, G AU - Salles G FAU - Dixit, V M AU - Dixit VM FAU - Rosenthal, A AU - Rosenthal A FAU - Schlegelberger, B AU - Schlegelberger B FAU - Morris, S W AU - Morris SW LA - eng SI - GENBANK/AF082283 SI - GENBANK/AF097732 GR - CA-27165/CA/NCI NIH HHS/United States PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, P.H.S. PL - United States TA - Nat Genet JT - Nature genetics JID - 9216904 RN - 0 (Adaptor Proteins, Signal Transducing) RN - 0 (B-Cell CLL-Lymphoma 10 Protein) RN - 0 (BCL10 protein, human) RN - 0 (NF-kappa B) RN - 0 (Neoplasm Proteins) RN - 9007-49-2 (DNA) RN - EC 3.4.22.- (Caspases) SB - IM MH - *Adaptor Proteins, Signal Transducing MH - Amino Acid Sequence MH - B-Cell CLL-Lymphoma 10 Protein MH - Binding Sites MH - Blotting, Northern MH - Caspases/*metabolism MH - Cell Death/genetics MH - Cell Line MH - Chromosomes, Human, Pair 1/genetics MH - Chromosomes, Human, Pair 14/genetics MH - DNA/chemistry/genetics MH - Female MH - Gene Expression Regulation, Neoplastic MH - Humans MH - In Situ Hybridization, Fluorescence MH - Lymphoma, B-Cell, Marginal Zone/*genetics MH - Male MH - Molecular Sequence Data MH - Mutation MH - NF-kappa B/metabolism MH - Neoplasm Proteins/chemistry/*genetics/metabolism MH - Protein Structure, Tertiary MH - Sequence Alignment MH - Sequence Analysis, DNA MH - Sequence Homology, Amino Acid MH - Tissue Distribution MH - Translocation, Genetic MH - Tumor Cells, Cultured EDAT- 1999/05/13 02:03 MHDA- 2001/03/23 10:01 CRDT- 1999/05/13 02:03 PHST- 1999/05/13 02:03 [pubmed] PHST- 2001/03/23 10:01 [medline] PHST- 1999/05/13 02:03 [entrez] AID - 10.1038/8767 [doi] PST - ppublish SO - Nat Genet. 1999 May;22(1):63-8. doi: 10.1038/8767.