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

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/js/lib/zoomerang.js