TEL/AML-1 dimerizes and is associated with a favorable outcome in childhood acute lymphoblastic leukemia
| Autor: | T W, McLean, S, Ringold, D, Neuberg, K, Stegmaier, R, Tantravahi, J, Ritz, H P, Koeffler, S, Takeuchi, J W, Janssen, T, Seriu, C R, Bartram, S E, Sallan, D G, Gilliland, T R, Golub |
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| Rok vydání: | 1996 |
| Předmět: |
Adult
Male Adolescent Oncogene Proteins Fusion Chromosomes Human Pair 21 Polymerase Chain Reaction Translocation Genetic Antineoplastic Combined Chemotherapy Protocols Proto-Oncogenes Biomarkers Tumor Humans Life Tables Cloning Molecular Child Chromosomes Human Pair 12 Infant DNA Neoplasm Precursor Cell Lymphoblastic Leukemia-Lymphoma Prognosis Survival Analysis Neoplasm Proteins Treatment Outcome Child Preschool Core Binding Factor Alpha 2 Subunit Female Dimerization Gene Deletion |
| Zdroj: | Blood. 88(11) |
| ISSN: | 0006-4971 |
| Popis: | Polymerase chain reaction-based screening of childhood acute lymphoblastic leukemia (ALL) samples showed that a TEL/AML1 fusion transcript was detected in 27% of all cases, representing the most common known gene rearrangement in childhood cancer. The TEL/AML1 fusion results from a t(12;21)(p13;q22) chromosomal translocation, but was undetectable at the routine cytogenetic level. TEL/AML1-positive patients had exclusively B-lineage ALL, and most patients were between the ages of 2 and 9 years at diagnosis. Only 3/89 (3.4%) adult ALL patients were TEL/AML1-positive. Most importantly, TEL/AML1-positive children had a significantly lower rate of relapse compared with TEL/AML1-negative patients (0/22 v 16/54, P = .004). Co-immunoprecipitation experiments demonstrated that TEL/AML-1 formed homodimers in vitro, and heterodimerized with the normal TEL protein when the two proteins were expressed together. The elucidation of the precise mechanism of transformation by TEL/AML1 and the role of TEL/AML1 testing in the treatment of childhood ALL will require additional studies. |
| Databáze: | OpenAIRE |
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