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

2009-08-01   Jean-Loup Huret 

1.Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France

Clinics and Pathology

Disease

Non Hodgkin lymphoma (NHL).

Epidemiology

Only one case available: a case of follicular lymphoma (FL) (Chen et al., 2001).

Genes Involved and Proteins

Gene name
BCL6 (B-Cell Lymphoma 6)
Location
3q27.3
Protein description
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 name
SRSF3 (serine/arginine-rich splicing factor 3)
Location
6p21.31
Protein description
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.

Bibliography

Pubmed IDLast YearTitleAuthors
125550642003Ambivalent role of BCL6 in cell survival and transformation.Albagli-Curiel O et al
115794682001Splicing factor SRP20 is a novel partner of BCL6 in a t(3;6)(q27;p21) translocation in transformed follicular lymphoma.Chen W et al

Summary

Fusion gene

SRSF3/BCL6 SRSF3 (6p21.31) BCL6 (3q27.3) M|SRSF3/BCL6 SRSF3 (6p21.31) BCL6 (3q27.3) M t(3;6)(q27;p21)|SRSF3/BCL6 SRSF3 (6p21.31) BCL6 (3q27.3) TIC

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.

Citation

Jean-Loup Huret

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

Atlas Genet Cytogenet Oncol Haematol. 2009-08-01

Online version: http://atlasgeneticsoncology.org/haematological/1336/t(3;6)(q27;p21)

External Links