t(6;14)(q25-27;q32) BCL11B/?

2008-07-01   Jean-Loup Huret  

1.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 IDYearTitleCitations
373369542023The role of transcription factors in shaping regulatory T cell identity.278
345012392021Biologic and Therapeutic Implications of Genomic Alterations in Acute Lymphoblastic Leukemia.195
333631422020Bcl11b/Ctip2 in Skin, Tooth, and Craniofacial System.168
314815362019Programming for T-lymphocyte fates: modularity and mechanisms.166
369691712023Transcriptional control of ILC identity.165
382126342024A new genomic framework to categorize pediatric acute myeloid leukemia.119
341033292021Enhancer Hijacking Drives Oncogenic BCL11B Expression in Lineage-Ambiguous Stem Cell Leukemia.117
323221902020Bcl11 Transcription Factors Regulate Cortical Development and Function.117
339361122021Epigenetic Dynamics in the Function of T-Lineage Regulatory Factor Bcl11b.108
383923442024The Role of Bcl11 Transcription Factors in Neurodevelopmental Disorders.108
388863782024Progressive senescence programs induce intrinsic vulnerability to aging-related female breast cancer.107
236444912013Proteomic and bioinformatic analysis of mammalian SWI/SNF complexes identifies extensive roles in human malignancy.102
224505362012Role of the transcription factor Bcl11b in development and lymphomagenesis.96
353313352022Human induced-T-to-natural killer cells have potent anti-tumour activities.93
337124722021The transcription factor Bcl11b promotes both canonical and adaptive NK cell differentiation.89
371159132023Bcl11b sustains multipotency and restricts effector programs of intestinal-resident memory CD8(+) T cells.85
377375602023BCL11B and the NuRD complex cooperatively guard T-cell fate and inhibit OPA1-mediated mitochondrial fusion in T cells.79
349631322022Transcription factors Bcl11a and Bcl11b are required for the production and differentiation of cortical projection neurons.79
364227062023International Consensus Classification of acute lymphoblastic leukemia/lymphoma.78
383583902024Regulation of hippocampal mossy fiber-CA3 synapse function by a Bcl11b/C1ql2/Nrxn3(25b+) pathway.78
391510202024Functional overlap of inborn errors of immunity and metabolism genes defines T cell metabolic vulnerabilities.78
360759762022Comparative roadmaps of reprogramming and oncogenic transformation identify Bcl11b and Atoh8 as broad regulators of cellular plasticity.74
356132742022FOXG1 sequentially orchestrates subtype specification of postmitotic cortical projection neurons.72
303741312018Bcl11b sets pro-T cell fate by site-specific cofactor recruitment and by repressing Id2 and Zbtb16.70
362896392022Postnatal Conditional Deletion of Bcl11b in Striatal Projection Neurons Mimics the Transcriptional Signature of Huntington's Disease.70
372939532023BCL11B expression in hepatocellular carcinoma relates to chemosensitivity and clinical prognosis.69
394339002024BMP suppresses Wnt signaling via the Bcl11b-regulated NuRD complex to maintain intestinal stem cells.67
284245912017Bcl11b-A Critical Neurodevelopmental Transcription Factor-Roles in Health and Disease.66
386323392024PU.1 and BCL11B sequentially cooperate with RUNX1 to anchor mSWI/SNF to poise the T cell effector landscape.64
251285522014The multifaceted roles of Bcl11b in thymic and peripheral T cells: impact on immune diseases.62
222649282012Transcriptional drivers of the T-cell lineage program.62
363763292022Symbiotic bacteria-dependent expansion of MR1-reactive T cells causes autoimmunity in the absence of Bcl11b.62
168277962006Radiation carcinogenesis in mouse thymic lymphomas.60
384723382024Pivotal role of BCL11B in the immune, hematopoietic and nervous systems: a review of the BCL11B-associated phenotypes from the genetic perspective.59
392664472024Developmental regulation of dermal adipose tissue by BCL11b.59
366265552023Evolution of nanobodies specific for BCL11A.57
295149172018Bcl11b, a novel GATA3-interacting protein, suppresses Th1 while limiting Th2 cell differentiation.55
380913912023Myeloid/natural killer (NK) cell precursor acute leukemia as a distinct leukemia type.54
388163542024ARID1B maintains mesenchymal stem cell quiescence via inhibition of BCL11B-mediated non-canonical Activin signaling.54
33876209202114q32 rearrangements deregulating BCL11B mark a distinct subgroup of T-lymphoid and myeloid immature acute leukemia.52
396137442024Cell-type specific, inducible and acute degradation of targeted protein in mice by two degron systems.52
316536912020Cell type-specific actions of Bcl11b in early T-lineage and group 2 innate lymphoid cells.52
388137292024Single-cell CRISPR screening characterizes transcriptional deregulation in T-cell acute lymphoblastic leukemia.52
292036432018The N-Terminal CCHC Zinc Finger Motif Mediates Homodimerization of Transcription Factor BCL11B.51
208240912010Increased expression of bcl11b leads to chemoresistance accompanied by G1 accumulation.50
282327442017The T-ALL related gene BCL11B regulates the initial stages of human T-cell differentiation.47
275868772016Identification of BCL11B as a regulator of adipogenesis.46
349466632021Easy Expression and Purification of Fluorescent N-Terminal BCL11B CCHC Zinc Finger Domain.46
294165012018Function of B-Cell CLL/Lymphoma 11B in Glial Progenitor Proliferation and Oligodendrocyte Maturation.44
330934452020BCL11B suppresses tumor progression and stem cell traits in hepatocellular carcinoma by restoring p53 signaling activity.44
335050232021AIM2 in regulatory T cells restrains autoimmune diseases.39
392957732024Dual role of BCL11B in T-cell malignancies.39
383065612024Role of PAX6, TRPA1, BCL11B, MCOLN2, CUX1, EMX1 in colorectal cancer and osteosarcoma.38
384958862024A Bcl11b(N797K) variant isolated from an immunodeficient patient inhibits early thymocyte development in mice.35
372493122023Autophagy Plays a Crucial Role in Ameloblast Differentiation.33
219126412011Genome-wide identification of Bcl11b gene targets reveals role in brain-derived neurotrophic factor signaling.31
300883732018Bcl11b Regulates IL-17 Through the TGF-β/Smad Pathway in HDM-Induced Asthma.30
259643702015Bcl11b is essential for group 2 innate lymphoid cell development.29
369185632023Screening of potential immune-related genes expressed during sepsis using gene sequencing technology.28
355633222022Generation of Inducible BCL11B Knockout in TAL1/LMO1 Transgenic Mouse T Cell Leukemia/Lymphoma Model.20
379863462023Differential expression of BCL11b and CDKN2A in CD30-positive peripheral T cell lymphoma: Retrospective study.20
262195582015Haploinsufficiency of Bcl11b suppresses the progression of ATM-deficient T cell lymphomas.20
366835252023A novel variant in BCL11B in an individual with neurodevelopmental delay: A case report.18
383175872024BCL11B promotes T-cell acute lymphoblastic leukaemia cell survival via the XRCC5/C11ORF21 axis.10
336635882021Inhibition of BCL11B induces downregulation of PTK7 and results in growth retardation and apoptosis in T-cell acute lymphoblastic leukemia.10
371203502023Emerging molecular subtypes and therapies in acute lymphoblastic leukemia.0
391736242024A single-cell transcriptomic census of mammalian olfactory epithelium aging.0
394873512024A mutant BCL11B-N440K protein interferes with BCL11A function during T lymphocyte and neuronal development.0
354215412022Oncogenic isoform switch of tumor suppressor BCL11B in adult T-cell leukemia/lymphoma.0
378609682024Clinico-biological refinement of BCL11B-related disorder and identification of an episignature: A series of 20 unreported individuals.0
265639952016Bcl11b: A New Piece to the Complex Puzzle of Amyotrophic Lateral Sclerosis Neuropathogenesis?0
394550002024BCL11b interacts with RNA and proteins involved in RNA processing and developmental diseases.0
398537402025Mechanisms of enhancer-driven oncogene activation.0
388201412024GFI1B specifies developmental potential of innate lymphoid cell progenitors in the lungs.0
344732342021BCL11B, the Cerberus of human leukemia.0
337124742021A natural killer's hike through epigenetic landscapes.0
374813192023Advances in Flow Cytometry for Mixed Phenotype and Ambiguous Leukemias.0
399762282025Double the Double: Revisiting BCL11B's Multimerization.0
321355952020[A case report of BCL11B mutation induced neurodevelopmental disorder and literature review].0
281104222017Interventions for Neurocognitive Dysfunction.0
388526382024Bimin Kang ameliorates the minimal persistent inflammation in allergic rhinitis by reducing BCL11B expression and regulating ILC2 plasticity.0
209695902010Critical roles of Bcl11b in T-cell development and maintenance of T-cell identity.0
384425742024LINC00158 modulates the function of BEAS-2B cells via targeting BCL11B and ameliorates OVA-LPS-induced severe asthma in mice models.0
231680722012BCL11B suppression does not influence CD34(+) cell differentiation and proliferation.0
366026022022BCL11B Upregulates the Expression of RelA in T Cells Stimulated with Staphylococcal Enterotoxin A.0
266984022016BCL11B/CTIP2 is highly expressed in GABAergic interneurons of the mouse somatosensory cortex.0
272878792016Bcl11b/Ctip2 is required for development of lingual papillae in mice.0
389646872024C(2)H(2)-type zinc-finger protein BCL11B suppresses avian Leukosis virus subgroup J replication by regulating apoptosis.0
288267742017Advances in basic and clinical immunology in 2016.0
286697332017BCL11B is frequently downregulated in HTLV-1-infected T-cells through Tax-mediated proteasomal degradation.0
370741862023The Majority of the Serine/Threonine Phosphorylation Sites in Bcl11b Protein Are Dispensable for the Differentiation of T Cells.0
316730692020BCL11B regulates MICA/B-mediated immune response by acting as a competitive endogenous RNA.0
256650792015Transcriptional regulation of innate and adaptive lymphocyte lineages.0
217565412011Down regulation of BCL11B expression inhibits proliferation and induces apoptosis in malignant T cells by BCL11B-935-siRNA.0
156687002005Disruption 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
356432262022An improved CUT&RUN method for regulation network reconstruction of low abundance transcription factor.0
173062242007Haploinsufficiency of Bcl11b for suppression of lymphomagenesis and thymocyte development.0
393894092024Genome-Wide Association Study Meta-Analysis of 9619 Cases With Tic Disorders.0
222451412012BCL11B tumor suppressor inhibits HDM2 expression in a p53-dependent manner.0
303521712019Alteration of Bcl11b upon stimulation of both the MAP kinase- and Gsk3-dependent signaling pathways in double-negative thymocytes.0

Article Bibliography

Pubmed IDLast YearTitleAuthors
87807431996Is t(6;14) a non-random translocation in childhood acute mixed lineage leukemia?Batanian JR et al
151042872004A novel t(6;14)(q25-q27;q32) in acute myelocytic leukemia involves the BCL11B gene.Bezrookove V et al
186566902008Acute mixed lineage leukemia and a t(6;14)(q25;q32) in two adults.Georgy M et al
2364166199014q32 translocations are associated with mixed-lineage expression in childhood acute leukemia.Hayashi Y et al
119204812002Abnormalities of chromosome bands 15q13-15 in childhood acute lymphoblastic leukemia.Heerema NA et al
23999101990Cytogenetic findings in 21 cases of peripheral T-cell lymphoma.Inwards DJ et al
161793742006Chromosomal translocations are associated with poor prognosis in chronic lymphocytic leukemia.Mayr C et al
27583951989Cytogenetics 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)