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t(3;7)(q27;q32) FRA7H/BCL6

Written2012-12Jean-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 1630

Clinics and Pathology

Disease Non Hodgkin lymphoma
Clinics Only 3 cases to date of t(3;7)(q27;q32): a B-cell lymphoma cell line established from a 55-year-old male patient with terminal diffuse large B-cell lymphoma (DLBCL), a t(11;14)(q23;q32) and a complex karyotype (Schneider et al., 2008); a 54-year-old male patient with stade IV splenic marginal zone B-cell lymphoma (SMZL) and a complex karyotype with +3 who's survival was 9 years+ (Boonstra et al., 2003); and a female patient with stade IV SMZL and a complex karyotype (Ott et al., 2000).

Genetics

The molecular event involving FRA7H and BCL6 has been uncovered only in the most recent report (Schneider et al., 2008).

Genes involved and Proteins

Gene NameBCL6 (B-Cell Lymphoma 6)
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 NameFRA7H (fragile site, aphidicolin type, common, fra(7)(q32.3))
Location 7q32.3
Note Fragile site, aphidicolin type, common.

Result of the chromosomal anomaly

Hybrid gene
Description The breakpoint at 7q32 maps within FRA7H; the breakpoint on chromosome 3 lies upstream of exon 1, out of both the major breakpoint region (MBR) and the alternative breakpoint regions (ABR). Hence, BCL6 is constitutively, albeit moderately, expressed (Schneider et al., 2008).
  

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
 
Splenic marginal zone lymphomas presenting with splenomegaly and typical immunophenotype are characterized by allelic loss in 7q31-32.
Boonstra R, Bosga-Bouwer A, van Imhoff GW, Krause V, Palmer M, Coupland RW, Dabbagh L, van den Berg E, van den Berg A, Poppema S.
Mod Pathol. 2003 Dec;16(12):1210-7.
PMID 14681321
 
Marginal zone B-cell lymphomas (MZBL) arising at different sites represent different biological entities.
Ott MM, Rosenwald A, Katzenberger T, Dreyling M, Krumdiek AK, Kalla J, Greiner A, Ott G, Muller-Hermelink HK.
Genes Chromosomes Cancer. 2000 Aug;28(4):380-6.
PMID 10862046
 
T(3;7)(q27;q32) fuses BCL6 to a non-coding region at FRA7H near miR-29.
Schneider B, Nagel S, Kaufmann M, Winkelmann S, Bode J, Drexler HG, MacLeod RA.
Leukemia. 2008 Jun;22(6):1262-6. Epub 2007 Nov 8.
PMID 17989715
 

Citation

This paper should be referenced as such :
Huret, JL
t(3;7)(q27;q32) FRA7H/BCL6
Atlas Genet Cytogenet Oncol Haematol. 2013;17(4):279-280.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0307q27q32ID1630.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;7)(q27;q32) FRA7H/BCL6

External links

arrayMap (UZH-SIB Zurich)
Mitelman databaset(3;7)(q27;q32) [Case List]    t(3;7)(q27;q32) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
 
Disease databaset(3;7)(q27;q32) FRA7H/BCL6
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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indexed on : Tue Nov 21 15:09:17 CET 2017


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