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FLASH GENE
Symbol BCL11B contributors: mct/shn - updated : 12-09-2017
HGNC name B-cell CLL/lymphoma 11B (zinc finger protein)
HGNC id 13222
PROTEIN
PHYSICAL PROPERTIES acid
STRUCTURE
motifs/domains
  • six C2H2-type zinc fingers
  • proline-rich domain
  • acidic regions
    • conjugated MetalloP
    HOMOLOGY
    interspecies ortholog to Bcl11b, Mus musculus
    homolog to Drosophila CG9650
    ortholog to Bcl11b, Rattus norvegicus
    ortholog to BCL11B, Pan troglodytes
    intraspecies homolog to BCL1A
    Homologene
    FAMILY
  • BCL11 family
    • CATEGORY transcription factor
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,cytosolic,granule
    intracellular,nucleus
    text
  • expressed by postmitotic granule cells in the developing as well as adult dentate gyrus
    • basic FUNCTION
  • a key regulator of both differentiation and survival during thymocyte development
  • a transcriptional regulator important for lymphopoiesis and previously associated with hematopoietic malignancies
  • involved in regulation of transcription, DNA-dependent
  • transcription factor required for the formation of the corticospinal tract
  • participates in the control of the interleukin-2 gene expression following activation through T-cell receptor
  • implicated in the remedy for DNA replication stress and maintenance of genomic integrity
  • plays essential roles in developmental processes of the immune and central nervous systems and skin
  • plays a crucial role in thymopoiesis and has been associated with hematopoietic malignancies
  • general transcriptional repressor for all long terminal repeats
  • its inhibition leads to apoptosis of malignant but not normal mature T cells
  • plays a major role in the development of corticospinal motor neuron axonal projections to the spinal cord
  • functions as a critical regulator of epithelial cell fate and differentiation during tooth morphogenesis
  • necessary role for ameloblast formation, location, differentiation, and maintenance
  • enhances NF-KappaB activation through up-regulation of MAP3K8 gene expression in T-lymphocytes
  • recruited to the CDKN1A gene promoter, silenced CDKN1A gene transcription through interactions with histone deacetylases and methyltransferases
  • constitutive CDKN1A gene suppressor that cooperates with SUV39H1 and histone methylation to silence the CDKN1A gene transcription
  • key transcriptional regulator of CDKN1A gene expression
  • recruits a repressive chromatin-modifying enzymatic complex to the HIV-1 promoter, and has an indirect effect on the cell cycle progression through CDKN1A promoter repression
  • constitutive CDKN1A gene suppressor that cooperates with SUV39H1 and histone methylation to silence the CDKN1A gene transcription
  • is necessary for T lineage commitment and required both to repress natural killer cell-associated genes and to down-regulate a battery of stem cell or progenitor cell genes at the pivotal stage of commitment
  • checkpoint during T cell development is Bcl11b-dependent
  • is a haploinsufficient tumor suppressor that collaborates with all major T-ALL oncogenic lesions in human thymocyte transformation
  • zinc finger transcription factor expressed in postmitotic neurons, playing a critical role during postnatal development of the dentate gyrus
  • plays a critical role in T-cell differentiation and proliferation
  • is a T-cell specific gene in hematopoiesis that begins expression during T-lineage commitment and is required for this process
  • unique roles in specific T cell populations, suggesting that its functions depend on cell type and activation state of the cell
  • is a new factor in the differentiation of type 2 innate lymphoid cells (ILC2s)
  • is more than just a T-cell-only transcription factor and sustains mature type 2 innate lymphoid cells (ILC2s) genetic and functional programs and lineage fidelity
  • adult expression of BCL11B is essential for survival, differentiation and functional integration of adult-born granule cell neurons, and BCL11B is required for survival of pre-existing mature neurons
  • during early tooth development, the transcription factor B&
    • 8209;cell CLL/lymphoma 11B (BCL11B) participates in dental epithelial cell proliferation and differentiation
  • serves a potentially important role in the regulation of ameloblast differentiation and enamel matrix protein expression
  • acts as a central intrinsic regulator of mammary epithelial stem cell quiescence and exhaustion and is necessary for long-term maintenance of the mammary gland
  • is essential for T-lineage commitment, particularly the repression of NK and myeloid potentials, and the induction of T-lineage genes, during the initial stages of human T-cell differentiation
    • CELLULAR PROCESS nucleotide, transcription, regulation
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule metal binding,
  • Zn2+
    • protein
  • Tat and HP1
  • NuRD complex (may be involved in transcriptional repression of BCL11B target genes and contribute to the function of BCL11B within a neuronal context) on the promoter of CDKN1C
  • in microglial cells, binds the HIV-1 promoter through CDKN1A, and recruits histone deacetylase (HDAC) and histone methyltransferase (HMT) activities to promote heterochromatic environment and HIV-1 transcriptional silencing
  • associates with the p300 coactivator in CD4+ T cells
  • recruits histone deacetylase (HDAC)1 and HDAC2 to promote local histone H3 deacetylation at the HIV-1 promoter region
  • BCL11B is a major downstream effector of FEZF2 in regulating the extension of axons toward subcortical targets and can rescue the axonal phenotype of FEZF2 mutants
  • cooperates with SUV39H1 and HDACs to suppress endogenous and Vpr-induced CDKN1A gene expression
  • important novel regulatory pathway in hippocampal development, by linking transcriptional functions of BCL11B to DSP, a molecule known to act on cell adhesion
  • is a dynamic regulator of epidermal proliferation and differentiation by integrating EGFR and NOTCH1 signaling
  • BCL11B is a major modulator of CDK9 activity and controls CDK9 function in physiological and pathological conditions
  • BCL11B binds to the SP6 promoter, and regulates SP6 gene expression through direct binding to the SP6 second promoter region
  • complex feedback regulatory network may exist between BCL11B and MSX2
  • BCL11B effects overlap with TCF3-dependent effects
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) IMD49
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral   translocation    
    translocated with TLX3 in t(5,14)(q35;q32.2) in B cell acute lymphoblastic leukemia
    tumoral   translocation    
    t(2;14)(q34;q32) (fusion of BCL11B and IKZF2) in adult T-cell leukemia/lymphoma (ATLL)
    tumoral     --low  
    is a key event in the multistep progression of ATLL (adult T-cell leukemia/lymphoma) leukemogenesis
    Susceptibility
    Variant & Polymorphism
    Candidate gene
  • may be considered a new candidate for targeted gene therapy in T-cell malignancies
    • Marker
    Therapy target
    ANIMAL & CELL MODELS
  • profound apoptosis in the thymus of neonatal Bcl11b-/- mice and adult mice show a block at the CD4-CD8- double-negative stage of thymocyte development