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t(3;6)(q27;p21) SRSF3/BCL6

Written2009-08Jean-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 1336
Note t(3;6)(q27;p21) has been described in a few cases where SFRS3/BCL6 rearrangement has not been ascertained, or where another hybrid gene has been uncovered.

Clinics and Pathology

Disease Non Hodgkin lymphoma (NHL).
Epidemiology Only one case available: a case of follicular lymphoma (FL) (Chen et al., 2001).

Cytogenetics

Cytogenetics Morphological The t(3;6) was accompanied with del(1q), del(7q), t(14;18)(q32;q21) and other anomalies.

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).
Gene NameSRSF3 (serine/arginine-rich splicing factor 3)
Location 6p21.31
Protein 164 amino acids (alternate splicing 124 aa); SFRS3 is a member of the serine- and arginine-rich (SR) protein family; comprise a RNA recognition motifs (RRM) (amino acids 10-83) and an SR domain (aa 86-164) according to Swiss-Prot; role in splicing of mRNA precursors; promote the export of some cellular mRNAs; possible role in cell cycle.

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
 
Splicing factor SRP20 is a novel partner of BCL6 in a t(3;6)(q27;p21) translocation in transformed follicular lymphoma.
Chen W, Itoyama T, Chaganti RS.
Genes Chromosomes Cancer. 2001 Nov;32(3):281-4.
PMID 11579468
 

Citation

This paper should be referenced as such :
Huret, JL
t(3;6)(q27;p21) SFRS3/BCL6
Atlas Genet Cytogenet Oncol Haematol. 2010;14(7):695-695.
Free journal version : [ pdf ]   [ DOI ]
On line version : http://AtlasGeneticsOncology.org/Anomalies/t0306q27p21ID1336.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;6)(q27;p21) SRSF3/BCL6

External links

arrayMap (UZH-SIB Zurich)SRSF3 (6p21.31) BCL6 (3q27.3)

SRSF3 (6p21.31) BCL6 (3q27.3)

SRSF3 (6p21.31) BCL6 (3q27.3)

Mitelman databaset(3;6)(q27;p21) [Case List]    t(3;6)(q27;p21) [Association List] Mitelman database (CGAP - NCBI)
arrayMap[select an item]
 
Mitelman databaseSRSF3/BCL6 [MCList]  SRSF3 (6p21.31) BCL6 (3q27.3)
Mitelman databaseSRSF3/BCL6 [MCList]  SRSF3 (6p21.31) BCL6 (3q27.3)
TICdbSRSF3/BCL6  SRSF3 (6p21.31) BCL6 (3q27.3)
 
Disease databaset(3;6)(q27;p21) SRSF3/BCL6
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
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