TEL/AML-1 dimerizes and is associated with a favorable outcome in childhood acute lymphoblastic leukemia

Autor: T. Seriu, Stephen E. Sallan, Donna Neuberg, C. R. Bartram, J. W. G. Janssen, D G Gilliland, Jerome Ritz, Ramana Tantravahi, T. W. Mclean, S. Takeuchi, S. Ringold, H. P. Koeffler, Todd R. Golub, Kimberly Stegmaier
Rok vydání: 1996
Předmět:
Zdroj: Blood. 88:4252-4258
ISSN: 1528-0020
0006-4971
DOI: 10.1182/blood.v88.11.4252.4252
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