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

Thomas W. McLean, Sarah Ringold, Donna Neuberg, Kimberly Stegmaier, Ramana Tantravahi, Jerome Ritz, H. Phillip Koeffler, Seisho Takeuchi, Johannes W.G. Janssen, Taku Seriu, Claus R. Bartram, Stephen E. Sallan, D. Gary Gilliland, Todd R. Golub

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303 Scopus citations

Abstract

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 (6/22 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.

Original languageEnglish
Pages (from-to)4252-4258
Number of pages7
JournalBlood
Volume88
Issue number11
DOIs
StatePublished - 1 Dec 1996

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