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

Pubmed IDYearTitleCitations
356409662022BCL6, a key oncogene, in the placenta, pre-eclampsia and endometriosis.181
394567512024B Cell Lymphoma 6 (BCL6): A Conserved Regulator of Immunity and Beyond.158
336639542021Bcl6-Mediated Transcriptional Regulation of Follicular Helper T cells (T(FH)).127
361661982022B-cell Lymphoma 6 (BCL6): From Master Regulator of Humoral Immunity to Oncogenic Driver in Pediatric Cancers.102
379671942023MKL/SRF and Bcl6 mutual transcriptional repression safeguards the fate and positioning of neocortical progenitor cells mediated by RhoA.95
317884712019Epigenetic Programing of B-Cell Lymphoma by BCL6 and Its Genetic Deregulation.81
237256552013New effector functions and regulatory mechanisms of BCL6 in normal and malignant lymphocytes.79
246984942014Breaking bad in the germinal center: how deregulation of BCL6 contributes to lymphomagenesis.75
383370962024Transcriptional programming of translation by BCL6 controls skeletal muscle proteostasis.73
346257922021Frequent mutations of FBXO11 highlight BCL6 as a therapeutic target in Burkitt lymphoma.70
386889342024Genomic deletion of Bcl6 differentially affects conventional dendritic cell subsets and compromises Tfh/Tfr/Th17 cell responses.69
366518762023Cell autonomous expression of BCL6 is required to maintain lineage identity of mouse CCR6+ ILC3s.68
355037212022The oncoprotein BCL6 enables solid tumor cells to evade genotoxic stress.68
319804862020Mutual inhibition between Prkd2 and Bcl6 controls T follicular helper cell differentiation.65
357661812022Developmental cell death of cortical projection neurons is controlled by a Bcl11a/Bcl6-dependent pathway.64
278815822017The Expanding Role of the BCL6 Oncoprotein as a Cancer Therapeutic Target.63
344645952021BCL6 controls contact-dependent help delivery during follicular T-B cell interactions.61
363776632022BCL6 is regulated by the MAPK/ELK1 axis and promotes KRAS-driven lung cancer.61
333935032021BCL6 confers KRAS-mutant non-small-cell lung cancer resistance to BET inhibitors.61
248797922014BCL6 controls Th9 cell development by repressing Il9 transcription.61
329115322021BCL6 maintains survival and self-renewal of primary human acute myeloid leukemia cells.60
268761842016Genome-wide Analysis Identifies Bcl6-Controlled Regulatory Networks during T Follicular Helper Cell Differentiation.60
184520902008Therapeutic targeting of the BCL6 oncogene for diffuse large B-cell lymphomas.59
324773382020Bcl6 Preserves the Suppressive Function of Regulatory T Cells During Tumorigenesis.59
372274912023The regulation of Tfh cell differentiation by β-hydroxybutyrylation modification of transcription factor Bcl6.58
290727032017BCL6 attenuates renal inflammation via negative regulation of NLRP3 transcription.57
313957412019Rationale for targeting BCL6 in MLL-rearranged acute lymphoblastic leukemia.52
398411442025BCL6 coordinates muscle mass homeostasis with nutritional states.51
258248192015BCL6 orchestrates Tfh cell differentiation via multiple distinct mechanisms.47
346981052021Evaluation of BCL6 and SIRT1 as Non-Invasive Diagnostic Markers of Endometriosis.47
332089432020Small-molecule-induced polymerization triggers degradation of BCL6.45
283565182017BCL6 promotes glioma and serves as a therapeutic target.45
360769402022miRNA-10a-5p Targeting the BCL6 Gene Regulates Proliferation, Differentiation and Apoptosis of Chicken Myoblasts.43
351000792022miR-30b-5p inhibits osteoblast differentiation through targeting BCL6.41
370600742023The malignancy suppression and ferroptosis facilitation of BCL6 in gastric cancer mediated by FZD7 repression are strengthened by RNF180/RhoC pathway.40
245712592014LMO2 and BCL6 are associated with improved survival in primary central nervous system lymphoma.39
373733542023Unveiling the Prognostic Significance of BCL6+/CD10+ Mantle Cell Lymphoma: Meta-Analysis of Individual Patients and Systematic Review.35
338074492021High-Grade B-Cell Lymphoma (HGBL) with MYC and BCL2 and/or BCL6 Rearrangements Is Predominantly BCL6-Rearranged and BCL6-Expressing in Taiwan.35
341350582021Bcl6-Independent In Vivo Development of Functional Type 1 Classical Dendritic Cells Supporting Tumor Rejection.31
354369842022B-cell lymphoma 6 alleviates nonalcoholic fatty liver disease in mice through suppression of fatty acid transporter CD36.30
220254992011Assembling defenses against therapy-resistant leukemic stem cells: Bcl6 joins the ranks.29
389378222024Characteristics and Clinical Value of MYC , BCL2, and BCL6 Rearrangement Detected by Next-generation Sequencing in DLBCL.27
394191752025A positive ReceptivaDx result for BCL6 does not correlate with abnormal ERA results or decreased expression of receptivity-associated markers: two sides of the endometrial receptivity coin in fertility evaluation and treatment.27
381847532024Non-IG::MYC in diffuse large B-cell lymphoma confers variable genomic configurations and MYC transactivation potential.26
364121012022BCL6-SPECC1L: A Novel Fusion Gene in Nasopharyngeal Carcinoma.25
354974982022BTB(BCL6) dimers as building blocks for reversible drug-induced protein oligomerization.23
367934352023Discovery of OICR12694: A Novel, Potent, Selective, and Orally Bioavailable BCL6 BTB Inhibitor.21
356536452022Improved Binding Affinity and Pharmacokinetics Enable Sustained Degradation of BCL6 In Vivo.20
196288152009Bcl6 mediates the development of T follicular helper cells.19
289294582018Backbone resonance assignment of the BCL6-BTB/POZ domain.10
225008402012Roles of BCL6 in normal and transformed germinal center B cells.0
289306822017Chemically Induced Degradation of the Oncogenic Transcription Factor BCL6.0
383352692024BCL6 is required for the thymic development of TCRαβ(+)CD8αα(+) intraepithelial lymphocyte lineage.0
387618042024Deficiency of CBL and CBLB ubiquitin ligases leads to hyper T follicular helper cell responses and lupus by reducing BCL6 degradation.0
384195812024BCL6 attenuates hyperoxia-induced lung injury by inhibiting NLRP3-mediated inflammation in fetal mouse.0
351251782022Predicting implantation failure: to BCL6 or not to BCL6.0
382899852024Pannexin-3 stabilizes the transcription factor Bcl6 in a channel-independent manner to protect against vascular oxidative stress.0
292627212018BCL6 as a therapeutic target for lymphoma.0
375565082023Targeting BCL6 in Gastrointestinal Stromal Tumor Promotes p53-Mediated Apoptosis to Enhance the Antitumor Activity of Imatinib.0
210836542011The role of BCL6 in lymphomas and routes to therapy.0
392087432024Discovery of novel BCL6-Targeting PROTACs with effective antitumor activities against DLBCL in vitro and in vivo.0
265668022015Mechanisms of action of BCL6 during germinal center B cell development.0
261084072015Mutual interaction between BCL6 and miRNAs contributing to the pathogenesis of various cancers.0
364419452022B-cell Lymphoma 6 Inhibitors: Current Advances and Prospects of Drug Development for Diffuse Large B-cell Lymphomas.0
387343432024BCL6 is a context-dependent mediator of the glioblastoma response to irradiation therapy.0
397163662025BCL6 Promotes Transcription of GPR61 to Suppress IL-1β-Induced Osteoarthritis Progression in C28/I2 Cells.0
160194572005Targeted somatic mutation of the BCL6 proto-oncogene and its impact on lymphomagenesis.0
391029892024BCL6 confers resistance to HDAC inhibitors in DLBCL.0
317321652019The Transcription Factor Tox2 Drives T Follicular Helper Cell Development via Regulating Chromatin Accessibility.0
384525142024Epidermal loss of Bcl6 exacerbates MC903-induced atopic dermatitis-like skin inflammation.0
150247212004Pathogenetic role of BCL6 translocation in B-cell non-Hodgkin's lymphoma.0
347741292022Structural basis of Apt48 inhibition of the BCL6 BTB domain.0
93731961997Significance of rearrangement of the BCL6 gene in B-cell lymphoid neoplasms.0
168156422007The BCL6 proto-oncogene: a leading role during germinal center development and lymphomagenesis.0
109539712000Internal deletions within the BCL6 gene in B-cell non-Hodgkin's lymphoma.0
205107342010BCL6: master regulator of the germinal center reaction and key oncogene in B cell lymphomagenesis.0
171429542006Pathogenetic and clinical implications of non-immunoglobulin ; BCL6 translocations in B-cell non-Hodgkin's lymphoma.0
352975662022Clinicopathological analysis of follicular lymphoma with BCL2, BCL6, and MYC rearrangements.0
393871122025A Small-Molecule BCL6 Inhibitor as an Anti-Proliferative Agent for Diffuse Large B-Cell Lymphoma.0
376344732023A small molecule BCL6 inhibitor as chemosensitizers in acute myeloid leukemia.0
346989642022FX1, a BCL6 inhibitor, reactivates BCL6 target genes and suppresses HTLV-1-infected T cells.0
381785932024Hypoxic stress caused apoptosis of MDBK cells by p53/BCL6-mitochondrial apoptosis pathway.0
123896162002Non-immunoglobulin/BCL6 gene fusion in diffuse large B-cell lymphoma: prognostic implications.0
126598622003Bcl6-dependent transcriptional repression by BAZF.0
255979952015Bcl6 promotes osteoblastogenesis through Stat1 inhibition.0
263190272016A Novel Non-Immunoglobulin (non-Ig)/BCL6 Translocation in Diffuse Large B-Cell Lymphoma Involving Chromosome 10q11.21 Loci and Review on Clinical Consequences of BCL6 Rearrangements.0
279212722017BCL6 locus is hypermethylated in angioimmunoblastic T-cell lymphoma.0
372793292023Foxp3 and Bcl6 deficiency synergistically induces spontaneous development of atopic dermatitis-like skin disease.0
331897302021Inhibition of the BCL6/miR-31/PKD1 axis attenuates oxidative stress-induced neuronal damage.0
386485642024Testing for t(3;8) in MYC/BCL6-rearranged large B-cell lymphoma identifies a high-risk subgroup with inferior survival.0
293979402018KSHV vIRF4 enhances BCL6 transcription via downregulation of IRF4 expression.0
353576542022BCL6 and the Notch pathway: a signaling axis leading to a novel druggable biotarget in triple negative breast cancer.0
313469872019Bcl6 Suppresses Cardiac Fibroblast Activation and Function via Directly Binding to Smad4.0
278562532017Discovery of high-affinity BCL6-binding peptide and its structure-activity relationship.0
124020372002Acetylation inactivates the transcriptional repressor BCL6.0
299722642019Bcl6 knockdown aggravates hypoxia injury in cardiomyocytes via the P38 pathway.0
349907522022An orally available small molecule BCL6 inhibitor effectively suppresses diffuse large B cell lymphoma cells growth in vitro and in vivo.0
117962282002Regulation of gene expression by the proto-oncogene BCL-6.0
75870611994BCL-6 and the molecular pathogenesis of B-cell lymphoma.0
331474672020Metabolic Reprogramming Induces Germinal Center B Cell Differentiation through Bcl6 Locus Remodeling.0

Article 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)