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

306 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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