t(3;6)(q27;p21) PIM1/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 diffuse large B-cell lymphoma (DLBCL) (Yoshida et al., 1999).

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
PIM1 (pim-1 oncogene)
Location
6p21.2
Protein description
404 amino acids; serine/threonine-protein kinase; regulated by hematopoietic cytokine receptors; synergy with c-MYC in cell proliferation and in apoptosis induction through an enhancement of the activation of caspase-3-like proteases; Cdc25A (cell cycle phosphatase) is a substrate for Pim-1

Result of the Chromosomal Anomaly

Description

5 PIM1 - 3 BCL6, but also 5 BCL6 - 3 PIM1; breakpoint in BCL6 between exon 1 and 2.

Article Bibliography

Pubmed IDLast YearTitleAuthors
125550642003Ambivalent role of BCL6 in cell survival and transformation.Albagli-Curiel O 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

Fusion gene

PIM1/BCL6 PIM1 (6p21.2) BCL6 (3q27.3) M t(3;6)(q27;p21)|PIM1/BCL6 PIM1 (6p21.2) BCL6 (3q27.3) TIC

Note

t(3;6)(q27;p21) has been described in a few cases were PIM1/BCL6 rearrangement has not been ascertained, or were another hybrid gene has been uncovered.

Citation

Jean-Loup Huret

t(3;6)(q27;p21) PIM1/BCL6

Atlas Genet Cytogenet Oncol Haematol. 2009-08-01

Online version: http://atlasgeneticsoncology.org/haematological/2088/t(3;6)(q27;p21)-pim1-bcl6