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

doi:10.1182/blood.v88.11.4252.4252

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

Icahn School of Medicine at Mount Sinai

Research output: Contribution to journal › Article › peer-review

308 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 language	English
Pages (from-to)	4252-4258
Number of pages	7
Journal	Blood
Volume	88
Issue number	11
DOIs	https://doi.org/10.1182/blood.v88.11.4252.4252
State	Published - 1 Dec 1996

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McLean, T. W., Ringold, S., Neuberg, D., Stegmaier, K., Tantravahi, R., Ritz, J., Koeffler, H. P., Takeuchi, S., Janssen, J. W. G., Seriu, T., Bartram, C. R., Sallan, S. E., Gilliland, D. G., & Golub, T. R. (1996). TEL/AML-1 dimerizes and is associated with a favorable outcome in childhood acute lymphoblastic leukemia. Blood, 88(11), 4252-4258. https://doi.org/10.1182/blood.v88.11.4252.4252

@article{67a22d81fe5244a1a08cf7097b5e5fec,

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

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.",

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

year = "1996",

month = dec,

day = "1",

doi = "10.1182/blood.v88.11.4252.4252",

language = "English",

volume = "88",

pages = "4252--4258",

journal = "Blood",

issn = "0006-4971",

publisher = "Elsevier B.V.",

number = "11",

}

McLean, TW, Ringold, S, Neuberg, D, Stegmaier, K, Tantravahi, R, Ritz, J, Koeffler, HP, Takeuchi, S, Janssen, JWG, Seriu, T, Bartram, CR, Sallan, SE, Gilliland, DG & Golub, TR 1996, 'TEL/AML-1 dimerizes and is associated with a favorable outcome in childhood acute lymphoblastic leukemia', Blood, vol. 88, no. 11, pp. 4252-4258. https://doi.org/10.1182/blood.v88.11.4252.4252

TY - JOUR

T1 - TEL/AML-1 dimerizes and is associated with a favorable outcome in childhood acute lymphoblastic leukemia

AU - McLean, Thomas W.

AU - Ringold, Sarah

AU - Neuberg, Donna

AU - Stegmaier, Kimberly

AU - Tantravahi, Ramana

AU - Ritz, Jerome

AU - Koeffler, H. Phillip

AU - Takeuchi, Seisho

AU - Janssen, Johannes W.G.

AU - Seriu, Taku

AU - Bartram, Claus R.

AU - Sallan, Stephen E.

AU - Gilliland, D. Gary

AU - Golub, Todd R.

PY - 1996/12/1

Y1 - 1996/12/1

N2 - 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.

AB - 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.

UR - https://www.scopus.com/pages/publications/0007055492

U2 - 10.1182/blood.v88.11.4252.4252

DO - 10.1182/blood.v88.11.4252.4252

M3 - Article

C2 - 8943861

AN - SCOPUS:0007055492

SN - 0006-4971

VL - 88

SP - 4252

EP - 4258

JO - Blood

JF - Blood

IS - 11

ER -

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

Abstract

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