The Hematopoietic Transcription Factors RUNX1 and ERG Prevent AML1-ETO Oncogene Overexpression and Onset of the Apoptosis Program in t(8;21) AMLs

Cell Rep. 2016 Nov 15;17(8):2087-2100. doi: 10.1016/j.celrep.2016.08.082.

Abstract

The t(8;21) acute myeloid leukemia (AML)-associated oncoprotein AML1-ETO disrupts normal hematopoietic differentiation. Here, we have investigated its effects on the transcriptome and epigenome in t(8,21) patient cells. AML1-ETO binding was found at promoter regions of active genes with high levels of histone acetylation but also at distal elements characterized by low acetylation levels and binding of the hematopoietic transcription factors LYL1 and LMO2. In contrast, ERG, FLI1, TAL1, and RUNX1 bind at all AML1-ETO-occupied regulatory regions, including those of the AML1-ETO gene itself, suggesting their involvement in regulating AML1-ETO expression levels. While expression of AML1-ETO in myeloid differentiated induced pluripotent stem cells (iPSCs) induces leukemic characteristics, overexpression increases cell death. We find that expression of wild-type transcription factors RUNX1 and ERG in AML is required to prevent this oncogene overexpression. Together our results show that the interplay of the epigenome and transcription factors prevents apoptosis in t(8;21) AML cells.

Keywords: AML1-ETO; RUNX1; acute myeloid leukemia; apoptosis; epigenome; iPSC.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acetylation
  • Apoptosis / genetics*
  • Base Sequence
  • Cell Line, Tumor
  • Cell Lineage / genetics
  • Cell Survival / genetics
  • Chromosomes, Human, Pair 21 / genetics
  • Chromosomes, Human, Pair 8 / genetics
  • Core Binding Factor Alpha 2 Subunit / metabolism*
  • Gene Knockdown Techniques
  • Genome, Human
  • Hematopoiesis / genetics*
  • Histone Deacetylases / metabolism
  • Humans
  • Leukemia, Myeloid, Acute / genetics*
  • Leukemia, Myeloid, Acute / pathology
  • Oncogene Proteins, Fusion / metabolism*
  • Oncogenes
  • Promoter Regions, Genetic
  • Protein Binding / genetics
  • RUNX1 Translocation Partner 1 Protein / metabolism*
  • Transcriptional Regulator ERG / metabolism
  • Translocation, Genetic*

Substances

  • AML1-ETO fusion protein, human
  • Core Binding Factor Alpha 2 Subunit
  • ERG protein, human
  • Oncogene Proteins, Fusion
  • RUNX1 Translocation Partner 1 Protein
  • Transcriptional Regulator ERG
  • Histone Deacetylases