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Blood, 15 December 2006, Vol. 108, No. 13, pp. 4198-4201. Prepublished online as a Blood First Edition Paper on August 22, 2006; DOI 10.1182/blood-2006-07-032953. This Article Full Text (PDF) Supplemental Tables and Figures All Versions of this Article: blood-2006-07-032953v1 108/13/4198    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Su, X.-Y. Articles by Penard-Lacronique, V. Search for Related Content PubMed PubMed Citation Articles by Su, X.-Y. Articles by Penard-Lacronique, V. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted July 5, 2006 Accepted August 1, 2006 HOX11L2/TLX3 is transcriptionally activated through T-cell regulatory elements downstream of BCL11B as a result of the t(5;14)(q35;q32) Xin-Ying Su, Veronique Della-Valle, Isabelle Andre-Schmutz, Claudie Lemercier, Isabelle Radford-Weiss, Paola Ballerini, Michel Lessard, Marina Lafage-Pochitaloff, Francine Mugneret, Roland Berger, Serge P Romana, Olivier Bernard, and Virginie Penard-Lacronique* INSERM0210; Hopital Necker, Paris INSERM E0210, Hopital Necker, Paris France INSERM U768, Hopital Necker-Enfants Malades, Paris, France INSERM U548, CEA-Grenoble/DRDC/ICH, Grenoble France INSERM E210 and Laboratoire de cytogenetique, Hopital Necker, Paris France Service d'hematologie, Hopital Trousseau, Paris, France Laboratoire d'Hematologie, Hopital de hautepierre, Strasbourg, France Laboratoire de cytogenetique,UMR599, Marseilles, France Laboratoire de cytogenetique, CHU de Dijon, France INSERM, E0210 ; Hopital Necker, Paris, France INSERM INSERM E0210; Hopital Necker, Paris * Corresponding author; email: penard{at}necker.fr. The t(5;14)(q35;q32) chromosomal translocation is specifically observed in up to 20% of childhood T-cell acute lymphoblastic leukemia (T-ALL). It affects the BCL11B/CTIP2 locus on chromosome 14 and the RANBP17-TLX3/HOX11L2 region on chromosome 5. It leads to ectopic activation of TLX3/HOX11L2. To investigate the reasons of the association between t(5;14) and T-ALL, we isolated the translocation breakpoints in eight t(5;14) patients. Sequence analyses did not involve recombinase activity in the genesis of the translocation. We used DNAse1 hypersensitive experiments to locate transcriptional regulatory elements downstream of BCL11B. By transient transfection experiments, two of the six regions demonstrated cis-activation properties in T-cells and were also effective on the TLX3 promoter. 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. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: K Buysse, A Crepel, B Menten, F Pattyn, F Antonacci, J A Veltman, L A Larsen, Z Tumer, A de Klein, I van de Laar, et al. Mapping of 5q35 chromosomal rearrangements within a genomically unstable region J. Med. Genet., October 1, 2008; 45(10): 672 - 678. [Abstract] [Full Text] [PDF] X. Su, V. Della-Valle, E. Delabesse, Z. Azgui, R. Berger, H. Merle-Beral, O. A Bernard, and F. Nguyen-Khac Transcriptional activation of the cardiac homeobox gene CSX1/NKX2-5 in a B-cell chronic lymphoproliferative disorder Haematologica, July 1, 2008; 93(7): 1081 - 1085. [Abstract] [Full Text] [PDF] C. Elso, X. Lu, S. Morrison, A. Tarver, H. Thompson, H. Thurkow, N. A. Yamada, and L. Stubbs Germline Translocations in Mice: Unique Tools for Analyzing Gene Function and Long-Distance Regulatory Mechanisms J Natl Cancer Inst Monographs, July 1, 2008; 2008(39): 91 - 95. [Abstract] [Full Text] [PDF] C. C. Tydell, E.-S. David-Fung, J. E. Moore, L. Rowen, T. Taghon, and E. V. Rothenberg Molecular Dissection of Prethymic Progenitor Entry into the T Lymphocyte Developmental Pathway J. Immunol., July 1, 2007; 179(1): 421 - 438. [Abstract] [Full Text] [PDF]

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