HOX11L2/TLX3 is transcriptionally activated through T-cell regulatory elements downstream of BCL11B as a result of the t(5;14)(q35;q32)
BLOOD
Authors: Su, Xin-Ying; Della-Valle, Veronique; Andre-Schmutz, Isabelle; Lemercier, Claudie; Radford-Weiss, Isabelle; Ballerini, Paola; Lessard, Michel; Lafage-Pochitaloff, Marina; Mugneret, Francine; Berger, Roland; Romana, Serge P.; Bernard, Olivier A.; Penard-Lacronique, Virginie
Abstract
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 BCL 11B/ 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 8 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, 2 of the 6 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. (Blood. 2006; 108:4198-4201) (c) 2006 by The American Society of Hematology.
A novel case of gamma delta T cell leukemia with recurrent genetic abnormalities accompanied by agranulocytosis
ANNALS OF HEMATOLOGY
Authors: Ichikawa, Satoshi; Fujiwara, Tohru; Saito, Kei; Fukuhara, Noriko; Yokoyama, Hisayuki; Hatta, Shunsuke; Onodera, Koichi; Onishi, Yasushi; Fujishima, Fumiyoshi; Ichinohasama, Ryo; Harigae, Hideo
Abstract