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Gene Review

TLX3  -  T-cell leukemia homeobox 3

Homo sapiens

Synonyms: HOX11L2, Homeobox protein Hox-11L2, RNX, T-cell leukemia homeobox protein 3
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Disease relevance of TLX3

  • A cryptic chromosome rearrangement, t(5;14)(q35.1;q32.2), recently identified in pediatric acute lymphoblastic leukemia (ALL), targets activation of TLX3 at 5q35.1 by juxtaposition with a region downstream of BCL11B at 14q32 [1].
  • This study further underscores that TLX3 expression in T-ALL is strongly associated with the presence of genomic rearrangements [2].
  • Finally, our data show that HOX11L2 expression was a suitable marker for minimal residual disease follow-up and was significantly associated with relapse (P =.02) [3].
  • Mutational analysis of the RNX gene in congenital central hypoventilation syndrome [4].
  • Based on this observation, we have carried out mutation screening of the RNX gene in a set of 13 patients affected with CCHS, 2 of whom showing association with Hirschsprung disease [4].

High impact information on TLX3

  • Our data indicate that the basis of the specific association between t(5;14) and T-ALL lies on the juxtaposition of TLX3 to long-range cis-activating regions active during T-cell differentiation [5].
  • By transient transfection experiments, 2 of the 6 regions demonstrated cis-activation properties in T cells and were also effective on the TLX3 promoter [5].
  • HOX11L2/TLX3 is transcriptionally activated through T-cell regulatory elements downstream of BCL11B as a result of the t(5;14)(q35;q32) [5].
  • Inclusion of HOXA-translocated cases in a general molecular portrait of 92 T-ALLs based on large-scale expression analysis shows that this rearrangement defines a new homogeneous subgroup, which shares common biologic networks with the TLX1- and TLX3-related cases [6].
  • Once CR was obtained, cumulative relapse rates were similar for IM, pre-alphabeta, and TCR+ T-ALL patients (P = .51), but were higher in HOX11L2 (83%) and SIL-TAL1 (82%) T-ALL patients compared with other genetic subgroups (48%; P = .05) [7].

Biological context of TLX3


Anatomical context of TLX3


Associations of TLX3 with chemical compounds


Regulatory relationships of TLX3

  • In this t(5;14) variant, NKX2-5 is expressed instead of TLX3 at both RNA and protein levels [1].

Other interactions of TLX3


Analytical, diagnostic and therapeutic context of TLX3


  1. The cardiac homeobox gene NKX2-5 is deregulated by juxtaposition with BCL11B in pediatric T-ALL cell lines via a novel t(5;14)(q35.1;q32.2). Nagel, S., Kaufmann, M., Drexler, H.G., MacLeod, R.A. Cancer Res. (2003) [Pubmed]
  2. Various types of rearrangements target TLX3 locus in T-cell acute lymphoblastic leukemia. Su, X.Y., Busson, M., Della Valle, V., Ballerini, P., Dastugue, N., Talmant, P., Ferrando, A.A., Baudry-Bluteau, D., Romana, S., Berger, R., Bernard, O.A. Genes Chromosomes Cancer (2004) [Pubmed]
  3. HOX11L2 expression defines a clinical subtype of pediatric T-ALL associated with poor prognosis. Ballerini, P., Blaise, A., Busson-Le Coniat, M., Su, X.Y., Zucman-Rossi, J., Adam, M., van den Akker, J., Perot, C., Pellegrino, B., Landman-Parker, J., Douay, L., Berger, R., Bernard, O.A. Blood (2002) [Pubmed]
  4. Mutational analysis of the RNX gene in congenital central hypoventilation syndrome. Matera, I., Bachetti, T., Cinti, R., Lerone, M., Gagliardi, L., Morandi, F., Motta, M., Mosca, F., Ottonello, G., Piumelli, R., Schober, J.G., Ravazzolo, R., Ceccherini, I. Am. J. Med. Genet. (2002) [Pubmed]
  5. HOX11L2/TLX3 is transcriptionally activated through T-cell regulatory elements downstream of BCL11B as a result of the t(5;14)(q35;q32). Su, X.Y., Della-Valle, V., Andre-Schmutz, I., Lemercier, C., Radford-Weiss, I., Ballerini, P., Lessard, M., Lafage-Pochitaloff, M., Mugneret, F., Berger, R., Romana, S.P., Bernard, O.A., Penard-Lacronique, V. Blood (2006) [Pubmed]
  6. HOXA genes are included in genetic and biologic networks defining human acute T-cell leukemia (T-ALL). Soulier, J., Clappier, E., Cayuela, J.M., Regnault, A., García-Peydró, M., Dombret, H., Baruchel, A., Toribio, M.L., Sigaux, F. Blood (2005) [Pubmed]
  7. Impact of TCR status and genotype on outcome in adult T-cell acute lymphoblastic leukemia: a LALA-94 study. Asnafi, V., Buzyn, A., Thomas, X., Huguet, F., Vey, N., Boiron, J.M., Reman, O., Cayuela, J.M., Lheritier, V., Vernant, J.P., Fiere, D., Macintyre, E., Dombret, H. Blood (2005) [Pubmed]
  8. t(5;14)/HOX11L2-positive T-cell acute lymphoblastic leukemia. A collaborative study of the Groupe Français de Cytogénétique Hématologique (GFCH). Berger, R., Dastugue, N., Busson, M., Van Den Akker, J., Pérot, C., Ballerini, P., Hagemeijer, A., Michaux, L., Charrin, C., Pages, M.P., Mugneret, F., Andrieux, J., Talmant, P., Hélias, C., Mauvieux, L., Lafage-Pochitaloff, M., Mozziconacci, M.J., Cornillet-Lefebvre, P., Radford, I., Asnafi, V., Bilhou-Nabera, C., Nguyen Khac, F., Léonard, C., Speleman, F., Poppe, B., Bastard, C., Taviaux, S., Quilichini, B., Herens, C., Grégoire, M.J., Cavé, H., Bernard, O.A. Leukemia (2003) [Pubmed]
  9. Assignment of the HOX11L2 gene to human chromosome band 5q35.1 and of its murine homolog to mouse chromosome bands 11A4-A5 by in situ hybridization. Cinti, R., Fava, M., Sancandi, M., Matera, I., Ravazzolo, R., Ceccherini, I. Cytogenet. Cell Genet. (2001) [Pubmed]
  10. Two dual-color split signal fluorescence in situ hybridization assays to detect t(5;14) involving HOX11L2 or CSX in T-cell acute lymphoblastic leukemia. van Zutven, L.J., Velthuizen, S.C., Wolvers-Tettero, I.L., van Dongen, J.J., Poulsen, T.S., MacLeod, R.A., Beverloo, H.B., Langerak, A.W. Haematologica (2004) [Pubmed]
  11. Gene expression signatures define novel oncogenic pathways in T cell acute lymphoblastic leukemia. Ferrando, A.A., Neuberg, D.S., Staunton, J., Loh, M.L., Huard, C., Raimondi, S.C., Behm, F.G., Pui, C.H., Downing, J.R., Gilliland, D.G., Lander, E.S., Golub, T.R., Look, A.T. Cancer Cell (2002) [Pubmed]
  12. Nuclear Factor Y Drives Basal Transcription of the Human TLX3, a Gene Overexpressed in T-Cell Acute Lymphocytic Leukemia. Borghini, S., Vargiolu, M., Di Duca, M., Ravazzolo, R., Ceccherini, I. Mol. Cancer Res. (2006) [Pubmed]
  13. Activation of HOX11L2 by juxtaposition with 3'-BCL11B in an acute lymphoblastic leukemia cell line (HPB-ALL) with t(5;14)(q35;q32.2). MacLeod, R.A., Nagel, S., Kaufmann, M., Janssen, J.W., Drexler, H.G. Genes Chromosomes Cancer (2003) [Pubmed]
  14. Transforming potential of the T-cell acute lymphoblastic leukemia-associated homeobox genes HOXA13, TLX1, and TLX3. Su, X., Drabkin, H., Clappier, E., Morgado, E., Busson, M., Romana, S., Soulier, J., Berger, R., Bernard, O.A., Lavau, C. Genes Chromosomes Cancer (2006) [Pubmed]
  15. NOTCH1 mutations in T-cell acute lymphoblastic leukemia: prognostic significance and implication in multifactorial leukemogenesis. Zhu, Y.M., Zhao, W.L., Fu, J.F., Shi, J.Y., Pan, Q., Hu, J., Gao, X.D., Chen, B., Li, J.M., Xiong, S.M., Gu, L.J., Tang, J.Y., Liang, H., Jiang, H., Xue, Y.Q., Shen, Z.X., Chen, Z., Chen, S.J. Clin. Cancer Res. (2006) [Pubmed]
  16. Clinical significance of HOX11L2 expression linked to t(5;14)(q35;q32), of HOX11 expression, and of SIL-TAL fusion in childhood T-cell malignancies: results of EORTC studies 58881 and 58951. Cavé, H., Suciu, S., Preudhomme, C., Poppe, B., Robert, A., Uyttebroeck, A., Malet, M., Boutard, P., Benoit, Y., Mauvieux, L., Lutz, P., Méchinaud, F., Grardel, N., Mazingue, F., Dupont, M., Margueritte, G., Pages, M.P., Bertrand, Y., Plouvier, E., Brunie, G., Bastard, C., Plantaz, D., Vande Velde, I., Hagemeijer, A., Speleman, F., Lessard, M., Otten, J., Vilmer, E., Dastugue, N. Blood (2004) [Pubmed]
  17. Assignment of the human homeobox 11-like 2 gene (HOX11L2) to chromosome 5q34-->q35 by radiation hybrid mapping. Lee-Kirsch, M.A., Engel, K., Paditz, E., Rösen-Wolff, A., Lee, Y.A., Gahr, M. Cytogenet. Cell Genet. (2001) [Pubmed]
  18. High Expression of the ETS Transcription Factor ERG Predicts Adverse Outcome in Acute T-Lymphoblastic Leukemia in Adults. Baldus, C.D., Burmeister, T., Martus, P., Schwartz, S., G??kbuget, N., Bloomfield, C.D., Hoelzer, D., Thiel, E., Hofmann, W.K. J. Clin. Oncol. (2006) [Pubmed]
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