Gene Symbol: Bcl11a
Description: B cell CLL/lymphoma 11A (zinc finger protein)
Alias: 2810047E18Rik, BCL-11A, Ctip1, D930021L15Rik, Evi9, Evi9a, Evi9b, Evi9c, mKIAA1809, B-cell lymphoma/leukemia 11A, B-cell CLL/lymphoma 11A, COUP-TF interacting protein 1, EVI-9, ecotropic viral integration site 9 protein, myeloid leukemia
Species: mouse
Products:     Bcl11a

Top Publications

  1. Nakamura T, Yamazaki Y, Saiki Y, Moriyama M, Largaespada D, Jenkins N, et al. Evi9 encodes a novel zinc finger protein that physically interacts with BCL6, a known human B-cell proto-oncogene product. Mol Cell Biol. 2000;20:3178-86 pubmed
    ..773 amino acids [aa]) contains two C(2)H(2)-type zinc finger motifs, a proline-rich region, and an acidic domain; Evi9b (486 aa) lacks the first zinc finger motif and part of the proline-rich region; Evi9c (239 aa) lacks all but the ..
  2. Nakamura T, Largaespada D, Shaughnessy J, Jenkins N, Copeland N. Cooperative activation of Hoxa and Pbx1-related genes in murine myeloid leukaemias. Nat Genet. 1996;12:149-53 pubmed
    ..These studies provide the first genetic evidence that Pbx1-related genes cooperate with Hox genes in leukaemia formation and identify a number of new murine myeloid leukaemia genes. ..
  3. Liu P, Jenkins N, Copeland N. A highly efficient recombineering-based method for generating conditional knockout mutations. Genome Res. 2003;13:476-84 pubmed
    ..This method should also facilitate the generation of knock-in mutations and transgene constructs, as well as expedite the analysis of regulatory elements and functional domains in or near genes. ..
  4. Leid M, Ishmael J, Avram D, Shepherd D, Fraulob V, Dolle P. CTIP1 and CTIP2 are differentially expressed during mouse embryogenesis. Gene Expr Patterns. 2004;4:733-9 pubmed
    Chicken ovalbumin upstream promoter transcription factor-interacting proteins 1 and 2 (CTIP1 and CTIP2) are related transcriptional regulatory proteins...
  5. Avram D, Fields A, Pretty On Top K, Nevrivy D, Ishmael J, Leid M. Isolation of a novel family of C(2)H(2) zinc finger proteins implicated in transcriptional repression mediated by chicken ovalbumin upstream promoter transcription factor (COUP-TF) orphan nuclear receptors. J Biol Chem. 2000;275:10315-22 pubmed
    Two novel and related C(2)H(2) zinc finger proteins that are highly expressed in the brain, CTIP1 and CTIP2 (COUP TF-interacting proteins 1 and 2, respectively), were isolated and shown to interact with all members of the chicken ..
  6. Xu J, Bauer D, Kerenyi M, Vo T, Hou S, Hsu Y, et al. Corepressor-dependent silencing of fetal hemoglobin expression by BCL11A. Proc Natl Acad Sci U S A. 2013;110:6518-23 pubmed publisher
    ..The transcription factor BCL11A is a critical modulator of hemoglobin switching and HbF silencing, yet the molecular mechanism through which ..
  7. Yu Y, Wang J, Khaled W, Burke S, Li P, Chen X, et al. Bcl11a is essential for lymphoid development and negatively regulates p53. J Exp Med. 2012;209:2467-83 pubmed publisher
    Transcription factors play important roles in lymphopoiesis. We have previously demonstrated that Bcl11a is essential for normal lymphocyte development in the mouse embryo...
  8. John A, Brylka H, Wiegreffe C, Simon R, Liu P, Jüttner R, et al. Bcl11a is required for neuronal morphogenesis and sensory circuit formation in dorsal spinal cord development. Development. 2012;139:1831-41 pubmed publisher
    ..Here we demonstrate that dorsal spinal neurons require the Krüppel-C(2)H(2) zinc-finger transcription factor Bcl11a for terminal differentiation and morphogenesis...
  9. Xu J, Peng C, Sankaran V, Shao Z, Esrick E, Chong B, et al. Correction of sickle cell disease in adult mice by interference with fetal hemoglobin silencing. Science. 2011;334:993-6 pubmed publisher
    ..Here, we show that the repressor BCL11A is required in vivo for silencing of ?-globin expression in adult animals, yet dispensable for red cell production...

More Information


  1. Sankaran V, Xu J, Ragoczy T, Ippolito G, Walkley C, Maika S, et al. Developmental and species-divergent globin switching are driven by BCL11A. Nature. 2009;460:1093-7 pubmed publisher
    ..We show that the expression of BCL11A, a repressor of human gamma-globin expression identified by genome-wide association studies, differs between mouse ..
  2. Yin B, Delwel R, Valk P, Wallace M, Loh M, Shannon K, et al. A retroviral mutagenesis screen reveals strong cooperation between Bcl11a overexpression and loss of the Nf1 tumor suppressor gene. Blood. 2009;113:1075-85 pubmed publisher
    ..One of these genes, Bcl11a, confers a growth advantage in cultured Nf1 mutant hematopoietic cells and causes early onset of leukemia of ..
  3. Senawong T, Peterson V, Leid M. BCL11A-dependent recruitment of SIRT1 to a promoter template in mammalian cells results in histone deacetylation and transcriptional repression. Arch Biochem Biophys. 2005;434:316-25 pubmed
    The B cell leukemia 11A protein (BCL11A/Evi9/CTIP1) has been implicated in hematopoietic cell development and malignancies...
  4. Liu P, Keller J, Ortiz M, Tessarollo L, Rachel R, Nakamura T, et al. Bcl11a is essential for normal lymphoid development. Nat Immunol. 2003;4:525-32 pubmed
    b>Bcl11a (also called Evi9) functions as a myeloid or B cell proto-oncogene in mice and humans, respectively. Here we show that Bcl11a is essential for postnatal development and normal lymphopoiesis...
  5. Ippolito G, Dekker J, Wang Y, Lee B, Shaffer A, Lin J, et al. Dendritic cell fate is determined by BCL11A. Proc Natl Acad Sci U S A. 2014;111:E998-1006 pubmed publisher
    ..Here we confirm an essential requirement for the BCL11A transcription factor in fetal pDC development, and demonstrate this lineage-specific requirement in the adult ..
  6. Chang Y, Stoykova A, Chowdhury K, Gruss P. Graded expression of Zfp462 in the embryonic mouse cerebral cortex. Gene Expr Patterns. 2007;7:405-12 pubmed
    ..This family includes transcription factor-encoding genes such as COUP-TF1, Emx2 and Pax6, which have been implicated in the process of cortical arealization. ..
  7. SMITH E, Luc S, Croney D, Woodworth M, Greig L, Fujiwara Y, et al. Strict in vivo specificity of the Bcl11a erythroid enhancer. Blood. 2016;128:2338-2342 pubmed publisher
    b>BCL11A, a repressor of human fetal (?-)globin expression, is required for immune and hematopoietic stem cell functions and brain development...
  8. Wu W, Hu Y, Li J, Zhu W, Ha T, Que L, et al. Silencing of Pellino1 improves post-infarct cardiac dysfunction and attenuates left ventricular remodelling in mice. Cardiovasc Res. 2014;102:46-55 pubmed publisher
    ..Our data demonstrate that Pellino1 plays an important role in the pathogenesis of MI. Targeting Pellino1 may ameliorate cardiac dysfunction and remodelling following MI. ..
  9. Roosjen M, McColl B, Kao B, Gearing L, Blewitt M, Vadolas J. Transcriptional regulators Myb and BCL11A interplay with DNA methyltransferase 1 in developmental silencing of embryonic and fetal ?-like globin genes. FASEB J. 2014;28:1610-20 pubmed publisher
    ..Following RNA interference (RNAi)-mediated knockdown of two key transcriptional regulators, Myb and BCL11A, we observed a derepression of ?-globin, measured by DsRed fluorescence and qRT-PCR (P<0.001)...
  10. Khaled W, Choon Lee S, Stingl J, Chen X, Raza Ali H, Rueda O, et al. BCL11A is a triple-negative breast cancer gene with critical functions in stem and progenitor cells. Nat Commun. 2015;6:5987 pubmed publisher
    ..Here, we report that the transcription factor BCL11A is overexpressed in TNBC including basal-like breast cancer (BLBC) and that its genomic locus is amplified in up ..
  11. Esteghamat F, Gillemans N, Bilic I, van den Akker E, Cantù I, van Gent T, et al. Erythropoiesis and globin switching in compound Klf1::Bcl11a mutant mice. Blood. 2013;121:2553-62 pubmed publisher
    B-cell lymphoma 11A (BCL11A) downregulation in human primary adult erythroid progenitors results in elevated expression of fetal ?-globin. Recent reports showed that BCL11A expression is activated by KLF1, leading to ?-globin repression...
  12. Kuwata T, Nakamura T. BCL11A is a SUMOylated protein and recruits SUMO-conjugation enzymes in its nuclear body. Genes Cells. 2008;13:931-40 pubmed publisher
    b>BCL11A/EVI9 is a zinc-finger protein predominantly expressed in brain and hematopoietic cells. Previous studies show that BCL11A is involved in acute myelomonocytic leukemia and chronic lymphoid leukemia in mouse and human, respectively...
  13. Sanosaka T, Namihira M, Asano H, Kohyama J, Aisaki K, Igarashi K, et al. Identification of genes that restrict astrocyte differentiation of midgestational neural precursor cells. Neuroscience. 2008;155:780-8 pubmed publisher
    ..Taken together, our data suggest that astrocyte differentiation of NPCs is regulated not only by DNA methylation but also by genes whose expression is controlled spatio-temporally during brain development. ..
  14. Wiegreffe C, Simon R, Peschkes K, Kling C, Strehle M, Cheng J, et al. Bcl11a (Ctip1) Controls Migration of Cortical Projection Neurons through Regulation of Sema3c. Neuron. 2015;87:311-25 pubmed publisher
    ..Here, we show that the transcription factor Bcl11a regulates polarity and migration of upper layer neurons...
  15. Paul A, Crow M, Raudales R, He M, Gillis J, Huang Z. Transcriptional Architecture of Synaptic Communication Delineates GABAergic Neuron Identity. Cell. 2017;171:522-539.e20 pubmed publisher
    ..This molecular genetic framework of neuronal identity integrates cell phenotypes along multiple axes and provides a foundation for discovering and classifying neuron types. ..
  16. Woodworth M, Greig L, Liu K, Ippolito G, Tucker H, Macklis J. Ctip1 Regulates the Balance between Specification of Distinct Projection Neuron Subtypes in Deep Cortical Layers. Cell Rep. 2016;15:999-1012 pubmed publisher
    ..Here, we report that Ctip1 controls precision of neocortical development by regulating subtype identity in deep-layer projection neurons...
  17. Powers A, Satija R. Single-cell analysis reveals key roles for Bcl11a in regulating stem cell fate decisions. Genome Biol. 2015;16:199 pubmed publisher
    Cell-cycle fluctuations drive significant transcriptomic heterogeneity in murine hematopoietic stem cells. Additionally, deletion of Bcl11a alters the regulation of hematopoietic stem cell quiescence, self-renewal, and fate choice.
  18. Wu X, Satpathy A, Kc W, Liu P, Murphy T, Murphy K. Bcl11a controls Flt3 expression in early hematopoietic progenitors and is required for pDC development in vivo. PLoS ONE. 2013;8:e64800 pubmed publisher
    b>Bcl11a is a transcription factor known to regulate lymphoid and erythroid development. Recent bioinformatic analysis of global gene expression patterns has suggested a role for Bcl11a in the development of dendritic cell (DC) lineages...
  19. Cánovas J, Berndt F, Sepúlveda H, Aguilar R, Veloso F, Montecino M, et al. The Specification of Cortical Subcerebral Projection Neurons Depends on the Direct Repression of TBR1 by CTIP1/BCL11a. J Neurosci. 2015;35:7552-64 pubmed publisher
    ..from layer 5 neurons in the mouse neocortex depends on the high levels of expression of the transcription factor CTIP1; CTIP1 is coexpressed with CTIP2 in neurons that project to subcerebral targets and with SATB2 in those that ..
  20. Jiang D, Liang J, Hodge J, Lu B, Zhu Z, Yu S, et al. Regulation of pulmonary fibrosis by chemokine receptor CXCR3. J Clin Invest. 2004;114:291-9 pubmed
    ..These data demonstrate a nonredundant role for CXCR3 in limiting tissue fibroproliferation and suggest that this effect may be mediated, in part, by the innate production of IFN-gamma following lung injury. ..
  21. Ideguchi M, Palmer T, Recht L, Weimann J. Murine embryonic stem cell-derived pyramidal neurons integrate into the cerebral cortex and appropriately project axons to subcortical targets. J Neurosci. 2010;30:894-904 pubmed publisher
    ..Thus, these data establish that ES-derived cortical projection neurons can integrate into anatomically relevant circuits. ..
  22. Benitez C, Qu K, Sugiyama T, Pauerstein P, Liu Y, Tsai J, et al. An integrated cell purification and genomics strategy reveals multiple regulators of pancreas development. PLoS Genet. 2014;10:e1004645 pubmed publisher
    ..regulators with corresponding mutant mice revealed that the transcription factors Etv1, Prdm16, Runx1t1 and Bcl11a are essential for pancreas development...
  23. Greig L, Woodworth M, Greppi C, Macklis J. Ctip1 Controls Acquisition of Sensory Area Identity and Establishment of Sensory Input Fields in the Developing Neocortex. Neuron. 2016;90:261-77 pubmed publisher
    ..Here, we report that the transcription factor Ctip1 functions in primary sensory areas to repress motor and activate sensory programs of gene expression, enabling ..
  24. Dias C, Estruch S, Graham S, McRae J, Sawiak S, Hurst J, et al. BCL11A Haploinsufficiency Causes an Intellectual Disability Syndrome and Dysregulates Transcription. Am J Hum Genet. 2016;99:253-74 pubmed publisher
    ..Here we report an ID syndrome caused by de novo heterozygous missense, nonsense, and frameshift mutations in BCL11A, encoding a transcription factor that is a putative member of the BAF swi/snf chromatin-remodeling complex...
  25. Luc S, Huang J, McEldoon J, Somuncular E, Li D, Rhodes C, et al. Bcl11a Deficiency Leads to Hematopoietic Stem Cell Defects with an Aging-like Phenotype. Cell Rep. 2016;16:3181-3194 pubmed publisher
    B cell CLL/lymphoma 11A (BCL11A) is a transcription factor and regulator of hemoglobin switching that has emerged as a promising therapeutic target for sickle cell disease and thalassemia...
  26. Saiki Y, Yamazaki Y, Yoshida M, Katoh O, Nakamura T. Human EVI9, a homologue of the mouse myeloid leukemia gene, is expressed in the hematopoietic progenitors and down-regulated during myeloid differentiation of HL60 cells. Genomics. 2000;70:387-91 pubmed
    b>Evi9, a common site of retroviral integration in BXH2 murine myeloid leukemias, encodes a C2H2 zinc finger protein and is overexpressed in these leukemic cells...
  27. Rashid M, Belmont J, Carpenter D, Turner C, Olson E. Neural-specific deletion of the focal adhesion adaptor protein paxillin slows migration speed and delays cortical layer formation. Development. 2017;144:4002-4014 pubmed publisher
  28. Chan C, Fulton J, Montiel Duarte C, Collins H, Bharti N, Wadelin F, et al. A signature motif mediating selective interactions of BCL11A with the NR2E/F subfamily of orphan nuclear receptors. Nucleic Acids Res. 2013;41:9663-79 pubmed publisher
    ..Here, we describe a novel signature motif (F/YSXXLXXL/Y) in the developmental regulator BCL11A that facilitates its selective interaction with members of the NR2E/F subfamily...