| Identity |
| Note | the translocation may be hidden, and can present as a del(11)(q23) |
| Clinics and Pathology |
| Disease | acute non lymphocytic leukemia (ANLL), acute lymphocytic leukemia (ALL) |
| Clinics | poorly known: a few cases, but without ascertainement of GAS7 involvement (a gene in 17p13); they are cases of MDS in transformation, ANLL, bi-phenotypic leukemia, and ALL; one the other hand, one case of t(11;17) with MLL and GAS7 involvementshas been described: a 13-year-old boy, with a M4 treatment-related ANLL and a karyotype with an apparent del(11)(q23) (a cryptic t(11;17)(q23;p13)); four months later the patient died |
| Genes involved and Proteins |
| Gene Name | MLL |
| Location | 11q23 |
| Dna / Rna | 21 exons, spanning over 100 kb; 13-15 kb mRNA |
| Protein | 431 kDa; contains two DNA binding motifs (a AT hook, and Zinc fingers), a DNA methyl transferase motif, a bromodomain; transcriptional regulatory factor; nuclear localisation |
| Gene Name | GAS7 |
| Location | 17p13 |
| Dna / Rna | genomic sequence 167 kb; 14 exons |
| Protein | expression in quiescent/terminally differenciated cells, especially in the brain |
| Result of the chromosomal anomaly |
| Description | 5' MLL-3' GAS7; breakpoint in MLL intron 8, in MLL bcr; GAS7 breakpoint upstream of exon 1; fusion of mLL exon 7 or exon 8 to GAS7 exon 2 |
| External links |
| Other database | t(11;17)(q23;p13) | Mitelman database (CGAP - NCBI) | |
| Other database | t(11;17)(q23;p13) | CancerChromosomes (NCBI) |
| To be noted |
| Additional cases are needed to delineate the epidemiology of this rare entity: you are welcome to submit a paper to our new Case Report section. |
| Bibliography |
| Ten novel 11q23 chromosomal partner sites. European 11q23 Workshop participants. |
| Harrison CJ, Cuneo A, Clark R, Johansson B, Lafage-Pochitaloff M, Mugneret F, Moorman AV, Secker-Walker LM |
| Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 1998 ; 12 (5) : 811-822. |
| PMID 9593286 |
| Detection of leukemia-associated MLL-GAS7 translocation early during chemotherapy with DNA topoisomerase II inhibitors. |
| Megonigal MD, Cheung NK, Rappaport EF, Nowell PC, Wilson RB, Jones DH, Addya K, Leonard DG, Kushner BH, Williams TM, Lange BJ, Felix CA |
| Proceedings of the National Academy of Sciences of the United States of America. 2000 ; 97 (6) : 2814-2819. |
| PMID 10706619 |
| Contributor(s) |
| Written | 06-2000 | Jean-Loup Huret |
| Citation |
| This paper should be referenced as such : |
| Huret JL . t(11;17)(q23;p13). Atlas Genet Cytogenet Oncol Haematol. June 2000 . URL : http://AtlasGeneticsOncology.org/Genes/t1117ID1137.html |
| © Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Mon Aug 11 21:20:49 2008 |
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