t(3;6)(q27;p21)

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

Seven cases available: 3 cases of follicular lymphoma (FL), 3 cases of diffuse large B-cell lymphoma (DLBCL), and one NHL not otherwise specified. Very few data available: 4 male / 2 female patients, aged 49-79 years (Ohno et al., 1994; Miura et al., 1996; Yoshida et al., 1999; Chen et al., 2001; Varga et al., 2001; Keller et al., 2006).

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).

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
168678732006Intrachromosomal rearrangement of chromosome 3q27: an under recognized mechanism of BCL6 translocation in B-cell non-Hodgkin lymphoma.Keller CE et al
89237871996A new non-random chromosomal translocation t(3;6)(q27;p21.3) associated with BCL6 rearrangement in two patients with non-Hodgkin's lymphoma.Miura I et al
80636121994Heterogeneity in B-cell neoplasms associated with rearrangement of the LAZ3 gene on chromosome band 3q27.Ohno H et al
113035272001Clustering of 1p36 breakpoints distal to 1p36.2 in hematological malignancies.Varga AE et al
106375101999Identification of heterologous translocation partner genes fused to the BCL6 gene in diffuse large B-cell lymphomas: 5'-RACE and LA - PCR analyses of biopsy samples.Yoshida S et al

Summary

Note

In only two cases were the hybrid genes uncovered: a PIM1/BCL6 was found in one case (Yoshida et al., 1999), and a SFRS3/BCL6 in another case (Chen et al., 2001). These cases will also be described separately.

Citation

Jean-Loup Huret

t(3;6)(q27;p21)

Atlas Genet Cytogenet Oncol Haematol. 2009-08-01

Online version: http://atlasgeneticsoncology.org/haematological/2155/t(3;6)(q27;p21)

External Links