AML1-ETO driven acute leukemia: Insights into pathogenesis and potential therapeutic approaches Journal Article


Authors: Hatlen, M. A.; Wang, L.; Nimer, S. D.
Article Title: AML1-ETO driven acute leukemia: Insights into pathogenesis and potential therapeutic approaches
Abstract: The AML1-ETO fusion transcription factor is generated by the t(8;21) translocation, which is present in approximately 4%-12% of adult and 12%-30% of pediatric acute myeloid leukemia (AML) patients. Both human and mouse models of AML have demonstrated that AML1-ETO is insufficient for leukemogenesis in the absence of secondary events. In this review, we discuss the pathogenetic insights that have been gained from identifying the various events that can cooperate with AML1-ETO to induce AML in vivo. We also discuss potential therapeutic strategies for t(8;21) positive AML that involve targeting the fusion protein itself, the proteins that bind to it, or the genes that it regulates. Recently published studies suggest that a targeted therapy for t(8;21) positive AML is feasible and may be coming sometime soon. © 2012 Higher Education Press and Springer-Verlag Berlin Heidelberg.
Keywords: leukemia; oncoprotein; acute granulocytic leukemia; gene translocation; genetics; mutation; leukemia, myeloid, acute; review; mouse; phenotype; animal; animals; mice; tumor markers, biological; genotype; transcription factor; chromosomes, human, pair 8; tumor marker; transgenic mouse; mice, transgenic; transcription factors; cell transformation, neoplastic; disease model; gene expression regulation; cell transformation; oncogene proteins, fusion; translocation, genetic; hematopoiesis; disease models, animal; chromosome 8; chromosome 21; core binding factor alpha 2 subunit; gene expression regulation, leukemic; chromosomes, human, pair 21; transcription factor runx1; mouse model; aml1-eto; t(8; 21); kasumi-1; pathway hits; cd34+; aml1 eto fusion protein, human; aml1-eto fusion protein, human; runx1 protein, mouse
Journal Title: Frontiers of Medicine
Volume: 6
Issue: 3
ISSN: 2095-0217
Publisher: Springer  
Date Published: 2012-09-01
Start Page: 248
End Page: 262
Language: English
DOI: 10.1007/s11684-012-0206-6
PROVIDER: scopus
PUBMED: 22875638
DOI/URL:
Notes: --- - "Export Date: 1 October 2012" - "Source: Scopus"
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  1. Stephen D Nimer
    347 Nimer
  2. Lan Wang
    14 Wang
  3. Megan A Hatlen
    14 Hatlen