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Gene Review

BCL11A  -  B-cell CLL/lymphoma 11A (zinc finger protein)

Homo sapiens

Synonyms: B-cell CLL/lymphoma 11A, B-cell lymphoma/leukemia 11A, BCL-11A, BCL11A-L, BCL11A-S, ...
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Disease relevance of BCL11A


High impact information on BCL11A

  • In 4 CLL cases with unmutated VH genes, a common minimal 3.5-Mb gain of 2p16 spanning the REL and BCL11A oncogenes was identified, implicating these genes in the pathogenesis of CLL [5].
  • BCL11A and BCL11B are transcriptional regulators important for lymphopoiesis and previously associated with hematopoietic malignancies [6].
  • This CpG island was associated with a Krüppel zinc finger gene (BCL11A), which is normally expressed at high levels only in fetal brain and in germinal center B-cells [1].
  • BCL11A was also highly homologous to another gene (BCL11B) on chromosome 14q32 [1].
  • However, transcriptional repression mediated by ARP1 acting through CTIP1 did not appear to involve recruitment of a trichostatin A-sensitive histone deacetylase(s) to the template, suggesting that this repression pathway may be distinct from that utilized by several other nuclear receptors [7].

Biological context of BCL11A


Anatomical context of BCL11A

  • Chicken ovalbumin upstream promoter transcription factor (COUP-TF)-interacting proteins 1 and 2 [CTIP1/Evi9/B cell leukaemia (Bcl) l1a and CTIP2/Bcl11b respectively] are highly related C(2)H(2) zinc finger proteins that are abundantly expressed in brain and the immune system, and are associated with immune system malignancies [10].
  • Furthermore, EVI9 was down-regulated during myeloid differentiation of HL60 cells induced by all-trans-retinoic acid, whereas the expression remained during monocytic differentiation induced by phorbol 12-myristate 13-acetate [11].
  • BCL11A-XL partitions into the nuclear matrix and colocalizes with BCL6 in nuclear paraspeckles [12].
  • Western blot data for BCL11A-XL expression coupled with data previously published for BCL6 indicates that these genes are expressed abundantly in germinal-center-derived B cells but that expression is extinguished upon terminal differentiation to the plasma cell stage [12].

Associations of BCL11A with chemical compounds

  • In both cases, BCL11A-mediated transcriptional repression is only minimally reversed by trichostatin A, suggesting the possible lack of involvement of class I or II histone deacetylases [8].

Physical interactions of BCL11A

  • SIRT1 was found to interact directly with BCL11A and was recruited to the promoter template in a BCL11A-dependent manner leading to transcriptional repression [8].
  • These results demonstrate that CTIP1 is a sequence-specific DNA binding protein and a bona fide transcriptional repressor that is capable of functioning independently of COUP-TF family members [10].

Other interactions of BCL11A

  • These findings define a role for SIRT1 in transcriptional repression mediated by BCL11A in mammalian cells [8].
  • COUP-TF (chicken ovalbumin upstream promoter transcription factor)-interacting protein 1 (CTIP1) is a sequence-specific DNA binding protein [10].
  • Genes recently shown to be involved in such translocations include BCL11A and MALT1 [13].

Analytical, diagnostic and therapeutic context of BCL11A

  • One cHL displayed selective amplification of the REL locus not affecting BCL11A; another case studied by FICTION and a cHL with cytogenetic 2p change investigated by fluorescence in situ hybridization showed signal patterns suggesting breakpoints in the region spanned by the REL probe [9].
  • Real-time quantitative PCR detected REL and BCL11A gene amplifications in the nine patients with gains at 2p13-p16 and only in one additional patient with normal chromosome 2 [14].


  1. The BCL11 gene family: involvement of BCL11A in lymphoid malignancies. Satterwhite, E., Sonoki, T., Willis, T.G., Harder, L., Nowak, R., Arriola, E.L., Liu, H., Price, H.P., Gesk, S., Steinemann, D., Schlegelberger, B., Oscier, D.G., Siebert, R., Tucker, P.W., Dyer, M.J. Blood (2001) [Pubmed]
  2. Lack of somatic hypermutation of IG V(H) genes in lymphoid malignancies with t(2;14)(p13;q32) translocation involving the BCL11A gene. Küppers, R., Sonoki, T., Satterwhite, E., Gesk, S., Harder, L., Oscier, D.G., Tucker, P.W., Dyer, M.J., Siebert, R. Leukemia (2002) [Pubmed]
  3. Epstein-Barr virus is integrated between REL and BCL-11A in American Burkitt lymphoma cell line (NAB-2). Luo, W.J., Takakuwa, T., Ham, M.F., Wada, N., Liu, A., Fujita, S., Sakane-Ishikawa, E., Aozasa, K. Lab. Invest. (2004) [Pubmed]
  4. A novel t(6;14)(q25-q27;q32) in acute myelocytic leukemia involves the BCL11B gene. Bezrookove, V., van Zelderen-Bhola, S.L., Brink, A., Szuhai, K., Raap, A.K., Barge, R., Beverstock, G.C., Rosenberg, C. Cancer Genet. Cytogenet. (2004) [Pubmed]
  5. Genome-wide analysis of DNA copy number changes and LOH in CLL using high-density SNP arrays. Pfeifer, D., Pantic, M., Skatulla, I., Rawluk, J., Kreutz, C., Martens, U.M., Fisch, P., Timmer, J., Veelken, H. Blood (2007) [Pubmed]
  6. BCL11B participates in the activation of IL2 gene expression in CD4+ T lymphocytes. Cismasiu, V.B., Ghanta, S., Duque, J., Albu, D.I., Chen, H.M., Kasturi, R., Avram, D. Blood (2006) [Pubmed]
  7. 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. Avram, D., Fields, A., Pretty On Top, K., Nevrivy, D.J., Ishmael, J.E., Leid, M. J. Biol. Chem. (2000) [Pubmed]
  8. BCL11A-dependent recruitment of SIRT1 to a promoter template in mammalian cells results in histone deacetylation and transcriptional repression. Senawong, T., Peterson, V.J., Leid, M. Arch. Biochem. Biophys. (2005) [Pubmed]
  9. Recurrent involvement of the REL and BCL11A loci in classical Hodgkin lymphoma. Martín-Subero, J.I., Gesk, S., Harder, L., Sonoki, T., Tucker, P.W., Schlegelberger, B., Grote, W., Novo, F.J., Calasanz, M.J., Hansmann, M.L., Dyer, M.J., Siebert, R. Blood (2002) [Pubmed]
  10. COUP-TF (chicken ovalbumin upstream promoter transcription factor)-interacting protein 1 (CTIP1) is a sequence-specific DNA binding protein. Avram, D., Fields, A., Senawong, T., Topark-Ngarm, A., Leid, M. Biochem. J. (2002) [Pubmed]
  11. 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. Saiki, Y., Yamazaki, Y., Yoshida, M., Katoh, O., Nakamura, T. Genomics (2000) [Pubmed]
  12. Functional studies of BCL11A: characterization of the conserved BCL11A-XL splice variant and its interaction with BCL6 in nuclear paraspeckles of germinal center B cells. Liu, H., Ippolito, G.C., Wall, J.K., Niu, T., Probst, L., Lee, B.S., Pulford, K., Banham, A.H., Stockwin, L., Shaffer, A.L., Staudt, L.M., Das, C., Dyer, M.J., Tucker, P.W. Mol. Cancer (2006) [Pubmed]
  13. The pathogenetic role of oncogenes deregulated by chromosomal translocation in B-cell malignancies. Dyer, M.J. Int. J. Hematol. (2003) [Pubmed]
  14. Clinicopathologic significance and prognostic value of chromosomal imbalances in diffuse large B-cell lymphomas. Beà, S., Colomo, L., López-Guillermo, A., Salaverria, I., Puig, X., Pinyol, M., Rives, S., Montserrat, E., Campo, E. J. Clin. Oncol. (2004) [Pubmed]
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