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t(3;9)(q27;p24) DMRT1/BCL6

Written2009-07Jean-Loup Huret
Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France
Updated2012-12Jean-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 1488

Clinics and Pathology

Disease Non Hodgkin lymphoma
Clinics The t(3;9)(q27;p24) was found in a gastric lymphoma, from a study with no individual data (Chen et al., 2006).

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 NameDMRT1 (doublesex and mab-3 related transcription factor 1)
Location 9p24.3
Protein Transcription factor that plays a key role in male sex determination in various animal species including homo sapiens (Herpin and Schartl, 2011). Susceptibility locus for testicular germ cell tumor (Turnbull et al., 2010).

Result of the chromosomal anomaly

Hybrid gene
Description The breakpoint in DMRT1 is between exons 3 and 4, and between exon 1 and 2 in BCL6. 5' DMRT1-3' BCL6.
  

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
 
High BCL6 expression predicts better prognosis, independent of BCL6 translocation status, translocation partner, or BCL6-deregulating mutations, in gastric lymphoma.
Chen YW, Hu XT, Liang AC, Au WY, So CC, Wong ML, Shen L, Tao Q, Chu KM, Kwong YL, Liang RH, Srivastava G.
Blood. 2006 Oct 1;108(7):2373-83. Epub 2006 Jun 13.
PMID 16772602
 
Dmrt1 genes at the crossroads: a widespread and central class of sexual development factors in fish.
Herpin A, Schartl M.
FEBS J. 2011 Apr;278(7):1010-9. doi: 10.1111/j.1742-4658.2011.08030.x. Epub 2011 Feb 25. (REVIEW)
PMID 21281449
 
Variants near DMRT1, TERT and ATF7IP are associated with testicular germ cell cancer.
Turnbull C, Rapley EA, Seal S, Pernet D, Renwick A, Hughes D, Ricketts M, Linger R, Nsengimana J, Deloukas P, Huddart RA, Bishop DT, Easton DF, Stratton MR, Rahman N; UK Testicular Cancer Collaboration.
Nat Genet. 2010 Jul;42(7):604-7. doi: 10.1038/ng.607. Epub 2010 Jun 13.
PMID 20543847
 

Citation

This paper should be referenced as such :
Huret, JL
t(3;9)(q27;p24) DMRT1/BCL6
Atlas Genet Cytogenet Oncol Haematol. 2013;17(4):281-282.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0309q27p24ID1488.html
History of this paper:
Huret, JL. t(3;9)(q27;p24). Atlas Genet Cytogenet Oncol Haematol. 2010;14(6):595-595.
http://documents.irevues.inist.fr/bitstream/handle/2042/44786/07-2009-t0309q27p24ID1488.pdf


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

Genes BCL6

Translocations implicated (Data extracted from papers in the Atlas)

 t(3;9)(q27;p24) DMRT1/BCL6

External links

arrayMap (UZH-SIB Zurich)DMRT1 (9p24.3) BCL6 (3q27.3)

DMRT1 (9p24.3) BCL6 (3q27.3)

Mitelman databaset(3;9)(q27;p24) [Case List]    t(3;9)(q27;p24) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
Mitelman databaseDMRT1/BCL6 [MCList]  DMRT1 (9p24.3) BCL6 (3q27.3)
Mitelman databaseDMRT1/BCL6 [MCList]  DMRT1 (9p24.3) BCL6 (3q27.3)
 
Disease databaset(3;9)(q27;p24) DMRT1/BCL6
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


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