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The cryptic chromosomal deletion del(11)(p12p13) as a new activation mechanism of LMO2 in pediatric T-cell acute lymphoblastic leukemia.
| Content Provider | Semantic Scholar |
|---|---|
| Author | Vlierberghe, Pieter Van Grotel, Martine Van Lee, Charles Helgason, Tryggvi Buijs-Gladdines, Jessica G. C. A. M. Passier, Monique M. C. J. Wering, Elisabeth R. Van Meijerink, Jules P. P. Pieters, Rob |
| Copyright Year | 2006 |
| Abstract | To identify new cytogenetic abnormalities associated with leukemogenesis or disease outcome, T-cell acute lymphoblastic leukemia (T-ALL) patient samples were analyzed by means of the array-comparative genome hybridization technique (array-CGH). Here, we report the identification of a new recurrent and cryptic deletion on chromosome 11 (del(11)(p12p13)) in about 4% (6/138) of pediatric T-ALL patients. Detailed molecular-cytogenetic analysis revealed that this deletion activates the LMO2 oncogene in 4 of 6 del(11)(p12p13)-positive T-ALL patients, in the same manner as in patients with an LMO2 translocation (9/138). The LMO2 activation mechanism of this deletion is loss of a negative regulatory region upstream of LMO2, causing activation of the proximal LMO2 promoter. LMO2 rearrangements, including this del(11)(p12p13) and t(11;14) (p13;q11) or t(7;11)(q35;p13), were found in the absence of other recurrent cytogenetic abnormalities involving HOX11L2, HOX11, CALM-AF10, TAL1, MLL, or MYC. LMO2 abnormalities represent about 9% (13/138) of pediatric T-ALL cases and are more frequent in pediatric T-ALL than appreciated until now. |
| File Format | PDF HTM / HTML |
| Alternate Webpage(s) | http://www.bloodjournal.org/content/bloodjournal/108/10/3520.full.pdf?sso-checked=true |
| PubMed reference number | 16873670v1 |
| Volume Number | 108 |
| Issue Number | 10 |
| Journal | Blood |
| Language | English |
| Access Restriction | Open |
| Subject Keyword | Adult T-Cell Lymphoma/Leukemia CDISC SDTM Disease Outcome Terminology Chromosome Aberrations Chromosome Deletion Chromosomes, Human, Pair 11 Congenital Abnormality Cytogenetic Analysis Cytogenetics DNA Sequence Rearrangement Deletion Mutation HOX11 protein KMT2A gene LMO2 gene Leukemia, B-Cell Leukemogenesis MLLT10 wt Allele Nucleic Acid Hybridization Oncogenes Patients Precursor Cell Lymphoblastic Leukemia Lymphoma Precursor T-Cell Lymphoblastic Leukemia-Lymphoma Proto-Oncogene Proteins c-myc Regulatory Sequences, Nucleic Acid TAL1 gene TLX3 wt Allele comparative genomic analysis lymphoblast t(11;14) |
| Content Type | Text |
| Resource Type | Article |
