t(3;6)(q27;q15) ?/BCL6

2012-11-01   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

Clinics

The t(3;6) was found in two cases of follicular lymphoma (Sanger et al., 1987; Sanchez-Izquierdo et al., 2001).

Prognosis

The patient reported by Sanger et al., 1987 was a 83-year-old female patient with a follicular mixed small clived and large cell lymphoma, which evolved to a diffuse mixed small lymphocytic and large cell lymphoma. She experienced persistent disease under treatment, refused further chemotherapy, and died two years after diagnosis.

Genes Involved and Proteins

Note
The partner of BCL6 in this translocation is unknown.
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). Role in germinal centers of lymphoid follicles. BCL6 prevents ATM and TP53 to induce apoptosis in response to DNA rearrangements such as somatic hypermutation and class switch recombination. Therefore essential for normal B cell development.

Highly cited references

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Bibliography

Pubmed IDLast YearTitleAuthors
125550642003Ambivalent role of BCL6 in cell survival and transformation.Albagli-Curiel O et al
115161112001Detection of translocations affecting the BCL6 locus in B cell non-Hodgkin's lymphoma by interphase fluorescence in situ hybridization.Sanchez-Izquierdo D et al
33151791987Initial and subsequent cytogenetic studies in malignant lymphoma.Sanger WG et al

Citation

Jean-Loup Huret

t(3;6)(q27;q15) ?/BCL6

Atlas Genet Cytogenet Oncol Haematol. 2012-11-01

Online version: http://atlasgeneticsoncology.org/haematological/1626/t(3;6)(q27;q15)