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The Runt Domain Transcription Factor, PEBP2/CBF, and its Involvement in Human Leukemia

  • Chapter
Oncogenes as Transcriptional Regulators

Part of the book series: Progress in Gene Expression ((PRGE))

Abstract

AML1 is the mammalian homolog of the Drosophila segmentation gene, runt, and was originally identified at the beakpoint of the chromosome translocation, t(8;21), associated with acute myeloid leukemia. Subsequently, AML1 was found to be a frequent target of chromosome translocations associated with several other types of human leukemia including that associated with t(12;21) lymphoblastic leukemia. The product of AML1 is the α subunit of transcription factor PEBP2/CBF. The DNA binding domain of AML1, termed the Runt domain, also serves as the additional function of interacting with its partner protein, the β subunit. The gene that encodes the β subunit is also the target of inversion of chromosome 16 [inv(16)], another chromosome anomaly associated with acute myeloid leukemia. This surprising observation strongly suggests that the two subunits indeed function together as a heterodimer and play an important role in hematopoiesis. Therefore, elucidation of the structure and function of the Runt domain factor, PEBP2/CBF, has become very important for our understanding of the pathogenesis of many types of human leukemia. The involvement of AML1 and the β subunit gene in various types of chromosome translocations has been reviewed recently (Nucifora and Rowley, 1995; Liu et al, 1995). Here we shall focus our discussion on reviewing the structure and the function of PEBP2/CBF and how these two subunits are structurally altered when they are activated as oncogenes by chromosome translocations. This subject has also been reviewed recently by several other investigators (Speck and Stacy, 1995; Hiebert et al, 1996 a; Ito, 1996).

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Authors
  1. Yoshiaki Ito
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  2. Suk-Chul Bae
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Editors and Affiliations

  1. Dept. of Biotechnology, Institut Pasteur, 28, rue du Dr. Roux, F-75724, Paris Cedex 15, France

    M. Yaniv

  2. CNRS UMR 146, Institut Curie, Section de Recherche, Centre Universitaire, F-91405, Orsay, France

    J. Ghysdael

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Ito, Y., Bae, SC. (1997). The Runt Domain Transcription Factor, PEBP2/CBF, and its Involvement in Human Leukemia. In: Yaniv, M., Ghysdael, J. (eds) Oncogenes as Transcriptional Regulators. Progress in Gene Expression. Birkhäuser, Basel. https://doi.org/10.1007/978-3-0348-8934-6_4

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  • DOI: https://doi.org/10.1007/978-3-0348-8934-6_4

  • Publisher Name: Birkhäuser, Basel

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  • Online ISBN: 978-3-0348-8934-6

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