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t(3;11)(q27;q23) POU2AF1/BCL6

Written2013-01Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424 BLOOD, BONE MARROW, & HEMATOPOIETIC SYS
Atlas_Id 2016

Clinics and Pathology

Disease Non Hodgkin lymphoma
Clinics Four cases to date: 3 cases of follicular lymphoma (FL) (Roumier et al., 2000) and one case of peripheral T-cell lymphoma (T-cell NHL) (Schlegelberger et al., 1994). This translocation has been also detected in Karpas 231 cell line (Galiègue-Zouitina et al., 1995; Galiègue-Zouitina et al., 1996). The 3 FL cases were female patients; the T-cell NHL case was a 38-year-old male patient.

Cytogenetics

Cytogenetics Morphological A complex karyotype was found in all the four cases. A t(14;18)(q32;q21) was found in the 3 FL cases, a trisomy 12 in two of these. Other known recurrent abnormalities were: + 7, del(6q), del(13q), i(17q), + 18, found in one case each.

Genes involved and Proteins

Note The gene rearrangement was determined in Roumier et al., 2000, Galiègue-Zouitina et al., 1995; Galiègue-Zouitina et al., 1996.
Gene Name BCL6
Location 3q27.3
Protein 706 amino acids; composed of a NH2-term BTB/POZ domain (amino acids 1-130 (32-99 according to Swiss-Prot) which mediates homodimerization and protein-protein interactions with other corepressors (including HDAC1 and NCOR2/SMRT to constitute a large repressing complex, another transcription repression domain (191-386), PEST sequences (300-417) with a KKYK motif (375-379), and six zinc finger at the C-term (518-541, 546-568, 574-596, 602-624, 630-652, 658-681), responsible for sequence specific DNA binding. Transcription repressor; recognizes the consensus sequence: TTCCT(A/C)GAA (Albagli-Curiel, 2003). Role in germinal centers of lymphoid follicles. BCL6 prevents ATM and TP53 to induce apoptosis in response to DNA rearrangements such as somatic hypermutation and class switch recombination. Therefore essential for normal B cell development.
Gene Name POU2AF1
Location 11q23.1
Note POU2AF1 is also named BOB1 or OBF1.
Protein B-cell specific transcriptional coactivator: involved in the transcription of immunoglobulin genes. Associates with POU2F1 (OCT1) or POU2F2 (OCT2).

Result of the chromosomal anomaly

Hybrid gene
Description 5' POU2AF1- 3' BCL6 and 5' BCL6 - 3' POU2AF1, leading to two fusion transcripts; breakpoint in BCL6 intron 1.
  
Fusion Protein
Description No fusion protein, but promoter exchange between both partner genes.
  

To be noted

Additional cases are needed to delineate the epidemiology of this rare entity:
you are welcome to submit a paper to our new Case Report section.

Bibliography

Ambivalent role of BCL6 in cell survival and transformation.
Albagli-Curiel O.
Oncogene. 2003 Jan 30;22(4):507-16.
PMID 12555064
 
Fusion of the LAZ3/BCL6 and BOB1/OBF1 genes by t(3; 11) (q27; q23) chromosomal translocation.
Galiegue-Zouitina S, Quief S, Hildebrand MP, Denis C, Lecocq G, Collyn-d'Hooghe M, Bastard C, Yuille M, Dyer MJ, Kerckaert JP.
C R Acad Sci III. 1995 Nov;318(11):1125-31.
PMID 8574789
 
The B cell transcriptional coactivator BOB1/OBF1 gene fuses to the LAZ3/BCL6 gene by t(3;11)(q27;q23.1) chromosomal translocation in a B cell leukemia line (Karpas 231).
Galieque Zouitina S, Quief S, Hildebrand MP, Denis C, Lecocq G, Collyn-d'Hooghe M, Bastard C, Yuille M, Dyer MJ, Kerckaert JP.
Leukemia. 1996 Apr;10(4):579-87.
PMID 8618432
 
FISH analysis with a YAC probe improves detection of LAZ3/BCL6 rearrangement in non-Hodgkin's lymphoma.
Roumier C, Galiegue-Zouitina S, Bastard C, Soenen V, Lai JL, Denis C, Buchonnet G, Kerckaert JP, Cosson A, Fenaux P, Preudhomme C.
Hematol J. 2000;1(2):117-25.
PMID 11920179
 
Recurrent chromosome abnormalities in peripheral T-cell lymphomas.
Schlegelberger B, Himmler A, Bartles H, Kuse R, Sterry W, Grote W.
Cancer Genet Cytogenet. 1994 Nov;78(1):15-22.
PMID 7987800
 

Citation

This paper should be referenced as such :
Huret, JL
t(3;11)(q27;q23) POU2AF1/BCL6
Atlas Genet Cytogenet Oncol Haematol. 2013;17(6):433-434.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0311q27q23ID2016.html


Other genes implicated (Data extracted from papers in the Atlas) [ 1 ]

Genes BCL6

Translocations implicated (Data extracted from papers in the Atlas)

 t(3;11)(q27;q23) POU2AF1/BCL6

External links

arrayMap (UZH-SIB Zurich)POU2AF1 (11q23.1) BCL6 (3q27.3)

POU2AF1 (11q23.1) BCL6 (3q27.3)

POU2AF1 (11q23.1) BCL6 (3q27.3)

Mitelman databaset(3;11)(q27;q23) [Case List]    t(3;11)(q27;q23) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
Mitelman databasePOU2AF1/BCL6 [MCList]  POU2AF1 (11q23.1) BCL6 (3q27.3)
Mitelman databasePOU2AF1/BCL6 [MCList]  POU2AF1 (11q23.1) BCL6 (3q27.3)
TICdbPOU2AF1/BCL6  POU2AF1 (11q23.1) BCL6 (3q27.3)
 
Disease databaset(3;11)(q27;q23) POU2AF1/BCL6
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
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