t(11;18)(q21;q21) BIRC3/MALT1

2001-09-01   Peter Marynen , Mathijs Baens 

1.Center for Human Genetics, Molecular Genetics, Flanders Interuniversity Institute for Biotechnology, Catholic University of Leuven, Leuven, Belgium

Clinics and Pathology

Disease

B-cell non Hodgkin lymphoma (NHL), marginal zone B-cell lymphoma of Mucosa-Associated Lymphoid Tissue ( ) -type

Phenotype stem cell origin

Marginal zone B-cells

Epidemiology

found in extranodal MZBCL or MALT-type (50 %), 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

Atlas Image
t(11;18) FISH - Baens Mathijs, Peter Marynen

Genes Involved and Proteins

Gene name
BIRC3 (baculoviral IAP repeat-containing 3)
Location
11q22.2
Protein description
68 kDa; 604 amino acids. Member of the inhibitor of apoptosis (IAP) protein family.
Gene name
MALT1 (mucosa associated lymphoid tissue lymphoma translocation gene 1)
Location
18q21.32
Protein description
92 kDa; 824 amino acids

Result of the Chromosomal Anomaly

Description

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.
Atlas Image
BIRC3/MALT1 fusion protein - Baens Mathijs, Peter Marynen

Description

N- term API2-MLT C-term fusion 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.

Highly cited references

Pubmed IDYearTitleCitations
228908382013High-resolution genomic profiling reveals clonal evolution and competition in gastrointestinal marginal zone B-cell lymphoma and its large cell variant.13
342038892021Genetic Characterization and Clinical Features of Helicobacter pylori Negative Gastric Mucosa-Associated Lymphoid Tissue Lymphoma.4
168049172006A novel fusion of the MALT1 gene and the microtubule-associated protein 4 (MAP4) gene occurs in diffuse large B-cell lymphoma.2
323485922020Establishment and characterization of a MALT lymphoma cell line carrying an API2-MALT1 translocation.1

Bibliography

Pubmed IDLast YearTitleAuthors
105238591999A novel gene, MALT1 at 18q21, is involved in t(11;18) (q21;q21) found in low-grade B-cell lymphoma of mucosa-associated lymphoid tissue.Akagi T et al
107513672000The product of the t(11;18), an API2-MLT fusion, marks nearly half of gastric MALT type lymphomas without large cell proliferation.Baens M et al
110660712000Structure 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 et al
109799682000Detection of t(11;18)(q21;q21) by interphase fluorescence in situ hybridization using API2 and MLT specific probes.Dierlamm J et al
109079432000The 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 et al

Summary

Fusion gene

BIRC3/MALT1 BIRC3 (11q22.2) MALT1 (18q21.32) M|BIRC3/MALT1 BIRC3 (11q22.2) MALT1 (18q21.32) M t(11;12;18)(q22;q13;q21) t(11;14;18)(q22;q32;q21) t(11;18)(q22;q21) t(14;18)(q32;q21) t(6;18;11)(q24;q21;q22)|BIRC3/MALT1 BIRC3 (11q22.2) MALT1 (18q21.32) TIC
Atlas Image
t(11;18)(q21;q21) BIRC3/MALT1 Diagram and G-banding - Courtesy Charles Bangs; R-banded karyotype - Courtesy Karolien Beel, Peter Meeus, Geneviu00e8ve Ameye and Lucienne Michaux

Citation

Peter Marynen ; Mathijs Baens

t(11;18)(q21;q21) BIRC3/MALT1

Atlas Genet Cytogenet Oncol Haematol. 2001-09-01

Online version: http://atlasgeneticsoncology.org/haematological/2022/t(11;18)(q21;q21)-birc3-malt1

External Links