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Access through your institution Buy or subscribe Acute myelogenous leukemia (AML) is a heterogeneous disease that is classified based on the morphology of the blasts and by the presence or

absence of specific cytogenetic abnormalities. Two of the more common cytogenetic abnormalities, t(8;21) and inv(16), are seen in cases with different morphologies; however, recent studies

have revealed that they have a common underlying pathogenesis and are therefore referred to collectively as the core binding factor leukemias (reviewed in Downing). These translocations

result in the alteration of the genes that encode the AML1/CBFβ core binding factor transcription complex, a critical regulator of normal hematopoiesis. AML1 was initially identified as the

target of the t(8;21) and was shown to encode a transcription factor that contained a central domain of approximately 100 amino acids that has a very high level of homology to the Drosophila

pair-rules protein runt. AML1 binds to the core enhancer DNA sequence, TGT/GGT, through its central runt homology domain (RHD) and its affinity for DNA is increased by heterodimerization

through the RHD with a second subunit called CBFβ. This heterodimeric transcription factor regulates the transcription of a large number of hematopoietic-specific targets, including but not

limited to myeloperoxidase, CSF1 receptor, the subunits of the T cell antigen receptor, interleukin (IL)-3, and GM-CSF. This is a preview of subscription content, access via your institution

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about institutional subscriptions * Read our FAQs * Contact customer support REFERENCES * Downing JR . The AML1-ETO chimeric transcription factor in acute myeloid leukemia: biology and

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transmission of a conditional AML1-ETO allele allows cre-mediated induction of AML1-ETO expression in multipotential hematopoietic progenitors in adult mice and results in the

immortalization of myeloid cells _Blood_ 1999 94: 54a (Abstr) Google Scholar  Download references AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Department of Pathology, St Jude

Children's Research Hospital, 332 North Lauderdale Street, Memphis, TN 38105, USA JR Downing Authors * JR Downing View author publications You can also search for this author inPubMed 

Google Scholar RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Downing, J. AML1/CBFβ transcription complex: its role in normal hematopoiesis and

leukemia. _Leukemia_ 15, 664–665 (2001). https://doi.org/10.1038/sj.leu.2402035 Download citation * Received: 09 October 2000 * Accepted: 30 October 2000 * Published: 01 May 2001 * Issue

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