title: Activation of HOX11L2 by juxtaposition with 3'-BCL11B in an acute lymphoblastic leukemia cell line (HPB-ALL) with t(5;14)(q35;q32.2).
abstract: Cytogenetic analysis of a pediatric T-cell acute lymphoblastic leukemia (ALL) cell line (HPB-ALL) revealed the cryptic t(5;14)(q35;q32.2), recently found in 15-20% pediatric T-ALL patients, with 5q35 and 14q32.2 breakpoints at 5'-HOX11L2 and 3'-BCL11B, respectively. Expression of both BCL11B, which is hematologically restricted to T cells, and HOX11L2 was detected, whereas adjacent genes at 5q35 (RANBP17) and 14q32 (VRK1, HSU88895) were not dysregulated. Apparently, t(5;14)(q35;q32.2) serves to activate HOX11L2 by juxtaposition with elements downstream of BCL11B, implying a novel dysregulatory mechanism comparable to TCRA/D or IGH juxtaposition. As well as providing molecular cytogenetic documentation of t(5;14) ALL, this report validates HPB-ALL cells for investigating this important new disease entity in vitro.
authors.affiliation: DSMZ, German Collection of Microorganisms and Cell Cultures, Department of Cell Cultures, Braunschweig, Germany. rml@dsmz.de
authors.forename: Roderick A F
authors.initials: RA
authors.lastname: MacLeod
authors.forename: Stefan
authors.initials: S
authors.lastname: Nagel
authors.forename: Maren
authors.initials: M
authors.lastname: Kaufmann
authors.forename: Johannes W G
authors.initials: JW
authors.lastname: Janssen
authors.forename: Hans G
authors.initials: HG
authors.lastname: Drexler
mesh_terms: D019457:Chromosome Breakage / Q000235:genetics; D002872:Chromosome Deletion; D002883:Chromosomes, Human, Pair 14 / Q000235:genetics; D002885:Chromosomes, Human, Pair 16 / Q000235:genetics; D002895:Chromosomes, Human, Pair 5 / Q000235:genetics; D020732:Cytogenetic Analysis / Q000379:methods; D015972:Gene Expression Regulation, Neoplastic / Q000235:genetics*; D015321:Gene Rearrangement / Q000235:genetics; D005801:Genes, Homeobox / Q000235:genetics; D018398:Homeodomain Proteins / Q000096:biosynthesis / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000235:genetics* / Q000473:pathology; D009363:Neoplasm Proteins / Q000096:biosynthesis / Q000235:genetics*; D015513:Oncogene Proteins / Q000096:biosynthesis / Q000235:genetics / Q000378:metabolism*; D011518:Proto-Oncogene Proteins; D014158:Transcription, Genetic / Q000235:genetics; D014178:Translocation, Genetic / Q000235:genetics*; D014407:Tumor Cells, Cultured
chemical_list: D018398:Homeodomain Proteins; D009363:Neoplasm Proteins; D015513:Oncogene Proteins; D011518:Proto-Oncogene Proteins; C489494:TLX3 protein, human; C071250:TLX1 protein, human