t(6;14)(q25-27;q32) BCL11B/?
2008-07-01 Jean-Loup Huret   Affiliation1.Genetics, Dept Medical Information, University of Poitiers; CHU Poitiers Hospital, F-86021 Poitiers, France
Clinics and Pathology
Disease
Haematological malignancies
Phenotype stem cell origin
The phenotype is variable, and the translocation may be heterogenous at the level of the genes involved; there was one acute lymphoblastic leukaemia (ALL), four biphenotypic acute leukaemias (BAL), two of which with expression of myeloid and T-cell markers, one acute myeloid leukaemia (AML), one chronic T-cell neoplasm, and one chronic lymphocytic leukaemia (B-CLL) Binet stage B.
Epidemiology
Only 8 cases to date (5M/2F); there was 3 children and 4 adults (ages were: 12, 14, 28, 41, 54, 62, ?, ?).
Prognosis
Survival data is available in only 5 cases; patients died: "shortly", at 17 months, 33 mths, 45 mths, and 112 mths after diagnosis.
Genes Involved and Proteins
Note
In only one of the above mentioned cases, BCL11B was detected as being involved in the translocation; the partner is unknown (Bezrookove et al., 2004). The case with BCL11B involvement was a case of M1-AML in a 54 year old male patient who died shortly after diagnosis. The t(6;14) was the sole anomaly. In other cases, data is missing concerning the genes implicated in the translocation.
Gene name
BCL11B (B-cell lymphoma/leukaemia 11B)
Location
14q32.2
Protein description
Kruppel-like zinc finger protein. Expressed in the immune system and in the brain. Regulates thymic differentiation and survival.
Highly cited references
| Pubmed ID | Year | Title | Citations |
|---|---|---|---|
| 37336954 | 2023 | The role of transcription factors in shaping regulatory T cell identity. | 278 |
| 34501239 | 2021 | Biologic and Therapeutic Implications of Genomic Alterations in Acute Lymphoblastic Leukemia. | 195 |
| 33363142 | 2020 | Bcl11b/Ctip2 in Skin, Tooth, and Craniofacial System. | 168 |
| 31481536 | 2019 | Programming for T-lymphocyte fates: modularity and mechanisms. | 166 |
| 36969171 | 2023 | Transcriptional control of ILC identity. | 165 |
| 38212634 | 2024 | A new genomic framework to categorize pediatric acute myeloid leukemia. | 119 |
| 34103329 | 2021 | Enhancer Hijacking Drives Oncogenic BCL11B Expression in Lineage-Ambiguous Stem Cell Leukemia. | 117 |
| 32322190 | 2020 | Bcl11 Transcription Factors Regulate Cortical Development and Function. | 117 |
| 33936112 | 2021 | Epigenetic Dynamics in the Function of T-Lineage Regulatory Factor Bcl11b. | 108 |
| 38392344 | 2024 | The Role of Bcl11 Transcription Factors in Neurodevelopmental Disorders. | 108 |
| 38886378 | 2024 | Progressive senescence programs induce intrinsic vulnerability to aging-related female breast cancer. | 107 |
| 23644491 | 2013 | Proteomic and bioinformatic analysis of mammalian SWI/SNF complexes identifies extensive roles in human malignancy. | 102 |
| 22450536 | 2012 | Role of the transcription factor Bcl11b in development and lymphomagenesis. | 96 |
| 35331335 | 2022 | Human induced-T-to-natural killer cells have potent anti-tumour activities. | 93 |
| 33712472 | 2021 | The transcription factor Bcl11b promotes both canonical and adaptive NK cell differentiation. | 89 |
| 37115913 | 2023 | Bcl11b sustains multipotency and restricts effector programs of intestinal-resident memory CD8(+) T cells. | 85 |
| 37737560 | 2023 | BCL11B and the NuRD complex cooperatively guard T-cell fate and inhibit OPA1-mediated mitochondrial fusion in T cells. | 79 |
| 34963132 | 2022 | Transcription factors Bcl11a and Bcl11b are required for the production and differentiation of cortical projection neurons. | 79 |
| 36422706 | 2023 | International Consensus Classification of acute lymphoblastic leukemia/lymphoma. | 78 |
| 38358390 | 2024 | Regulation of hippocampal mossy fiber-CA3 synapse function by a Bcl11b/C1ql2/Nrxn3(25b+) pathway. | 78 |
| 39151020 | 2024 | Functional overlap of inborn errors of immunity and metabolism genes defines T cell metabolic vulnerabilities. | 78 |
| 36075976 | 2022 | Comparative roadmaps of reprogramming and oncogenic transformation identify Bcl11b and Atoh8 as broad regulators of cellular plasticity. | 74 |
| 35613274 | 2022 | FOXG1 sequentially orchestrates subtype specification of postmitotic cortical projection neurons. | 72 |
| 30374131 | 2018 | Bcl11b sets pro-T cell fate by site-specific cofactor recruitment and by repressing Id2 and Zbtb16. | 70 |
| 36289639 | 2022 | Postnatal Conditional Deletion of Bcl11b in Striatal Projection Neurons Mimics the Transcriptional Signature of Huntington's Disease. | 70 |
| 37293953 | 2023 | BCL11B expression in hepatocellular carcinoma relates to chemosensitivity and clinical prognosis. | 69 |
| 39433900 | 2024 | BMP suppresses Wnt signaling via the Bcl11b-regulated NuRD complex to maintain intestinal stem cells. | 67 |
| 28424591 | 2017 | Bcl11b-A Critical Neurodevelopmental Transcription Factor-Roles in Health and Disease. | 66 |
| 38632339 | 2024 | PU.1 and BCL11B sequentially cooperate with RUNX1 to anchor mSWI/SNF to poise the T cell effector landscape. | 64 |
| 25128552 | 2014 | The multifaceted roles of Bcl11b in thymic and peripheral T cells: impact on immune diseases. | 62 |
| 22264928 | 2012 | Transcriptional drivers of the T-cell lineage program. | 62 |
| 36376329 | 2022 | Symbiotic bacteria-dependent expansion of MR1-reactive T cells causes autoimmunity in the absence of Bcl11b. | 62 |
| 16827796 | 2006 | Radiation carcinogenesis in mouse thymic lymphomas. | 60 |
| 38472338 | 2024 | Pivotal role of BCL11B in the immune, hematopoietic and nervous systems: a review of the BCL11B-associated phenotypes from the genetic perspective. | 59 |
| 39266447 | 2024 | Developmental regulation of dermal adipose tissue by BCL11b. | 59 |
| 36626555 | 2023 | Evolution of nanobodies specific for BCL11A. | 57 |
| 29514917 | 2018 | Bcl11b, a novel GATA3-interacting protein, suppresses Th1 while limiting Th2 cell differentiation. | 55 |
| 38091391 | 2023 | Myeloid/natural killer (NK) cell precursor acute leukemia as a distinct leukemia type. | 54 |
| 38816354 | 2024 | ARID1B maintains mesenchymal stem cell quiescence via inhibition of BCL11B-mediated non-canonical Activin signaling. | 54 |
| 33876209 | 2021 | 14q32 rearrangements deregulating BCL11B mark a distinct subgroup of T-lymphoid and myeloid immature acute leukemia. | 52 |
| 39613744 | 2024 | Cell-type specific, inducible and acute degradation of targeted protein in mice by two degron systems. | 52 |
| 31653691 | 2020 | Cell type-specific actions of Bcl11b in early T-lineage and group 2 innate lymphoid cells. | 52 |
| 38813729 | 2024 | Single-cell CRISPR screening characterizes transcriptional deregulation in T-cell acute lymphoblastic leukemia. | 52 |
| 29203643 | 2018 | The N-Terminal CCHC Zinc Finger Motif Mediates Homodimerization of Transcription Factor BCL11B. | 51 |
| 20824091 | 2010 | Increased expression of bcl11b leads to chemoresistance accompanied by G1 accumulation. | 50 |
| 28232744 | 2017 | The T-ALL related gene BCL11B regulates the initial stages of human T-cell differentiation. | 47 |
| 27586877 | 2016 | Identification of BCL11B as a regulator of adipogenesis. | 46 |
| 34946663 | 2021 | Easy Expression and Purification of Fluorescent N-Terminal BCL11B CCHC Zinc Finger Domain. | 46 |
| 29416501 | 2018 | Function of B-Cell CLL/Lymphoma 11B in Glial Progenitor Proliferation and Oligodendrocyte Maturation. | 44 |
| 33093445 | 2020 | BCL11B suppresses tumor progression and stem cell traits in hepatocellular carcinoma by restoring p53 signaling activity. | 44 |
| 33505023 | 2021 | AIM2 in regulatory T cells restrains autoimmune diseases. | 39 |
| 39295773 | 2024 | Dual role of BCL11B in T-cell malignancies. | 39 |
| 38306561 | 2024 | Role of PAX6, TRPA1, BCL11B, MCOLN2, CUX1, EMX1 in colorectal cancer and osteosarcoma. | 38 |
| 38495886 | 2024 | A Bcl11b(N797K) variant isolated from an immunodeficient patient inhibits early thymocyte development in mice. | 35 |
| 37249312 | 2023 | Autophagy Plays a Crucial Role in Ameloblast Differentiation. | 33 |
| 21912641 | 2011 | Genome-wide identification of Bcl11b gene targets reveals role in brain-derived neurotrophic factor signaling. | 31 |
| 30088373 | 2018 | Bcl11b Regulates IL-17 Through the TGF-β/Smad Pathway in HDM-Induced Asthma. | 30 |
| 25964370 | 2015 | Bcl11b is essential for group 2 innate lymphoid cell development. | 29 |
| 36918563 | 2023 | Screening of potential immune-related genes expressed during sepsis using gene sequencing technology. | 28 |
| 35563322 | 2022 | Generation of Inducible BCL11B Knockout in TAL1/LMO1 Transgenic Mouse T Cell Leukemia/Lymphoma Model. | 20 |
| 37986346 | 2023 | Differential expression of BCL11b and CDKN2A in CD30-positive peripheral T cell lymphoma: Retrospective study. | 20 |
| 26219558 | 2015 | Haploinsufficiency of Bcl11b suppresses the progression of ATM-deficient T cell lymphomas. | 20 |
| 36683525 | 2023 | A novel variant in BCL11B in an individual with neurodevelopmental delay: A case report. | 18 |
| 38317587 | 2024 | BCL11B promotes T-cell acute lymphoblastic leukaemia cell survival via the XRCC5/C11ORF21 axis. | 10 |
| 33663588 | 2021 | Inhibition of BCL11B induces downregulation of PTK7 and results in growth retardation and apoptosis in T-cell acute lymphoblastic leukemia. | 10 |
| 37120350 | 2023 | Emerging molecular subtypes and therapies in acute lymphoblastic leukemia. | 0 |
| 39173624 | 2024 | A single-cell transcriptomic census of mammalian olfactory epithelium aging. | 0 |
| 39487351 | 2024 | A mutant BCL11B-N440K protein interferes with BCL11A function during T lymphocyte and neuronal development. | 0 |
| 35421541 | 2022 | Oncogenic isoform switch of tumor suppressor BCL11B in adult T-cell leukemia/lymphoma. | 0 |
| 37860968 | 2024 | Clinico-biological refinement of BCL11B-related disorder and identification of an episignature: A series of 20 unreported individuals. | 0 |
| 26563995 | 2016 | Bcl11b: A New Piece to the Complex Puzzle of Amyotrophic Lateral Sclerosis Neuropathogenesis? | 0 |
| 39455000 | 2024 | BCL11b interacts with RNA and proteins involved in RNA processing and developmental diseases. | 0 |
| 39853740 | 2025 | Mechanisms of enhancer-driven oncogene activation. | 0 |
| 38820141 | 2024 | GFI1B specifies developmental potential of innate lymphoid cell progenitors in the lungs. | 0 |
| 34473234 | 2021 | BCL11B, the Cerberus of human leukemia. | 0 |
| 33712474 | 2021 | A natural killer's hike through epigenetic landscapes. | 0 |
| 37481319 | 2023 | Advances in Flow Cytometry for Mixed Phenotype and Ambiguous Leukemias. | 0 |
| 39976228 | 2025 | Double the Double: Revisiting BCL11B's Multimerization. | 0 |
| 32135595 | 2020 | [A case report of BCL11B mutation induced neurodevelopmental disorder and literature review]. | 0 |
| 28110422 | 2017 | Interventions for Neurocognitive Dysfunction. | 0 |
| 38852638 | 2024 | Bimin Kang ameliorates the minimal persistent inflammation in allergic rhinitis by reducing BCL11B expression and regulating ILC2 plasticity. | 0 |
| 20969590 | 2010 | Critical roles of Bcl11b in T-cell development and maintenance of T-cell identity. | 0 |
| 38442574 | 2024 | LINC00158 modulates the function of BEAS-2B cells via targeting BCL11B and ameliorates OVA-LPS-induced severe asthma in mice models. | 0 |
| 23168072 | 2012 | BCL11B suppression does not influence CD34(+) cell differentiation and proliferation. | 0 |
| 36602602 | 2022 | BCL11B Upregulates the Expression of RelA in T Cells Stimulated with Staphylococcal Enterotoxin A. | 0 |
| 26698402 | 2016 | BCL11B/CTIP2 is highly expressed in GABAergic interneurons of the mouse somatosensory cortex. | 0 |
| 27287879 | 2016 | Bcl11b/Ctip2 is required for development of lingual papillae in mice. | 0 |
| 38964687 | 2024 | C(2)H(2)-type zinc-finger protein BCL11B suppresses avian Leukosis virus subgroup J replication by regulating apoptosis. | 0 |
| 28826774 | 2017 | Advances in basic and clinical immunology in 2016. | 0 |
| 28669733 | 2017 | BCL11B is frequently downregulated in HTLV-1-infected T-cells through Tax-mediated proteasomal degradation. | 0 |
| 37074186 | 2023 | The Majority of the Serine/Threonine Phosphorylation Sites in Bcl11b Protein Are Dispensable for the Differentiation of T Cells. | 0 |
| 31673069 | 2020 | BCL11B regulates MICA/B-mediated immune response by acting as a competitive endogenous RNA. | 0 |
| 25665079 | 2015 | Transcriptional regulation of innate and adaptive lymphocyte lineages. | 0 |
| 21756541 | 2011 | Down regulation of BCL11B expression inhibits proliferation and induces apoptosis in malignant T cells by BCL11B-935-siRNA. | 0 |
| 15668700 | 2005 | Disruption of the BCL11B gene through inv(14)(q11.2q32.31) results in the expression of BCL11B-TRDC fusion transcripts and is associated with the absence of wild-type BCL11B transcripts in T-ALL. | 0 |
| 35643226 | 2022 | An improved CUT&RUN method for regulation network reconstruction of low abundance transcription factor. | 0 |
| 17306224 | 2007 | Haploinsufficiency of Bcl11b for suppression of lymphomagenesis and thymocyte development. | 0 |
| 39389409 | 2024 | Genome-Wide Association Study Meta-Analysis of 9619 Cases With Tic Disorders. | 0 |
| 22245141 | 2012 | BCL11B tumor suppressor inhibits HDM2 expression in a p53-dependent manner. | 0 |
| 30352171 | 2019 | Alteration of Bcl11b upon stimulation of both the MAP kinase- and Gsk3-dependent signaling pathways in double-negative thymocytes. | 0 |
Article Bibliography
| Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|
| 8780743 | 1996 | Is t(6;14) a non-random translocation in childhood acute mixed lineage leukemia? | Batanian JR et al |
| 15104287 | 2004 | A novel t(6;14)(q25-q27;q32) in acute myelocytic leukemia involves the BCL11B gene. | Bezrookove V et al |
| 18656690 | 2008 | Acute mixed lineage leukemia and a t(6;14)(q25;q32) in two adults. | Georgy M et al |
| 2364166 | 1990 | 14q32 translocations are associated with mixed-lineage expression in childhood acute leukemia. | Hayashi Y et al |
| 11920481 | 2002 | Abnormalities of chromosome bands 15q13-15 in childhood acute lymphoblastic leukemia. | Heerema NA et al |
| 2399910 | 1990 | Cytogenetic findings in 21 cases of peripheral T-cell lymphoma. | Inwards DJ et al |
| 16179374 | 2006 | Chromosomal translocations are associated with poor prognosis in chronic lymphocytic leukemia. | Mayr C et al |
| 2758395 | 1989 | Cytogenetics of childhood acute nonlymphocytic leukemia. | Raimondi SC et al |
Citation
Jean-Loup Huret
t(6;14)(q25-27;q32) BCL11B/?
Atlas Genet Cytogenet Oncol Haematol. 2008-07-01
Online version: http://atlasgeneticsoncology.org/haematological/1324/t(6;14)(q25-27;q32)-bcl11b
