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| | BIRC3 protein - Baens Mathijs, Peter Marynen |
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| Description | 68 kDa; 604 amino acids. The protein contains three 'baculovirus inhibition of apoptosis' (BIR) repeats, a caspace recruitment domain or CARD and a carboxyterminal 'Zn-binding' RING domain. |
| Expression | wide, most abundantly in kidney, liver, small intestine and lung |
| Function | Member of the 'inhibitor of apoptosis' (IAP) protein family. Binds to the tumor necrosis factor receptor via its associated factors TRAF1 and TRAF2 and inhibits apoptosis, probably by interfering with activation of caspaces. |
| Homology | with Baculovirus IAP proteins, Drosophila DIAP1 and DIAP2 |
| Entity | t(11;18)(q21;q21) / marginal zone B-cell lymphoma ofMALT-type --> BIRC3 - MALT1 |
| Disease | B-cell non Hodgkin lymphoma (NHL); marginal zone B-cell lymphoma (MZBCL) of mucosa-associated lymphoid tissue (MALT); found in extranodal MZBCL or MALT-type, absent in splenic and nodal MZBCL |
| Prognosis | For gastric MALT-type lymphomas, t(11;18) is a clonal marker for resistance to Helicobacter pylori eradication therapy and antigen independent growth |
| Cytogenetics | t(11;18) is frequently associated with deletions affecting 3' API2 on chromosome 11 or 5' MALT1 on chromosome 18. |
| Hybrid/Mutated Gene | 5' API2 on chromosome 11q21 translocated on chromosome 18 in frame with 3' MALT1. Deletions often exclude the expression of the reciprocal 5' MLT ' API2 3' transcript. |
| Abnormal Protein | All MALT-type lymphomas reported with a t(11;18) express an 'in frame' API2-MLT fusion protein with consistently the three BIR domains of API2 fused to the caspace p20 domain and VDJ4-like domain of MLT. |
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| BIRC3/MALT1 fusion protein - Baens Mathijs, Peter Marynen |
| Oncogenesis | Transient expression of the API2-MLT fusion protein acitvates an NF-KB luciferase reporter construct, suggesting the involvement of this signaling cascade in MALT lymphomagenesis |
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| The apoptosis inhibitor gene API2 and a novel 18q gene, MLT, are recurrently rearranged in the t(11;18)(q21;q21) associated with mucosa-associated lymphoid tissue lymphomas. |
| Dierlamm J, Baens M, Wlodarska I, Stefanova-Ouzounova M, Hernandez JM, Hossfeld DK, De Wolf-Peeters C, Hagemeijer A, Van den Berghe H, Marynen P |
| Blood. 1999 ; 93 (11) : 3601-3609. |
| PMID 10339464 |
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| The product of the t(11;18), an API2-MLT fusion, marks nearly half of gastric MALT type lymphomas without large cell proliferation. |
| Baens M, Maes B, Steyls A, Geboes K, Marynen P, De Wolf-Peeters C |
| The American journal of pathology. 2000 ; 156 (4) : 1433-1439. |
| PMID 10751367 |
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| The product of the t(11;18), an API2-MLT fusion, is an almost exclusive finding in marginal zone cell lymphoma of extranodal MALT-type. |
| Maes B, Baens M, Marynen P, De Wolf-Peeters C |
| Annals of oncology : official journal of the European Society for Medical Oncology / ESMO. 2000 ; 11 (5) : 521-526. |
| PMID 10907943 |
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| Detection of t(11;18)(q21;q21) by interphase fluorescence in situ hybridization using API2 and MLT specific probes. |
| Dierlamm J, Baens M, Stefanova-Ouzounova M, Hinz K, Wlodarska I, Maes B, Steyls A, Driessen A, Verhoef G, Gaulard P, Hagemeijer A, Hossfeld DK, De Wolf-Peeters C, Marynen P |
| Blood. 2000 ; 96 (6) : 2215-2218. |
| PMID 10979968 |
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| Structure of the MLT gene and molecular characterization of the genomic breakpoint junctions in the t(11;18)(q21;q21) of marginal zone B-cell lymphomas of MALT type. |
| Baens M, Steyls A, Dierlamm J, De Wolf-Peeters C, Marynen P |
| Genes, chromosomes & cancer. 2000 ; 29 (4) : 281-291. |
| PMID 11066071 |
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