The Polycomb complex PRC2 supports aberrant self-renewal in a mouse model of MLL-AF9;Nras(G12D) acute myeloid leukemia

Oncogene. 2013 Feb 14;32(7):930-8. doi: 10.1038/onc.2012.110. Epub 2012 Apr 2.

Abstract

The Trithorax and Polycomb groups of chromatin regulators are critical for cell-lineage specification during normal development; functions that often become deregulated during tumorigenesis. As an example, oncogenic fusions of the Trithorax-related protein mixed lineage leukemia (MLL) can initiate aggressive leukemias by altering the transcriptional circuitry governing hematopoietic cell differentiation, a process that requires multiple epigenetic pathways to implement. Here we used shRNA screening to identify chromatin regulators uniquely required in a mouse model of MLL-fusion acute myeloid leukemia, which revealed a role for the Polycomb repressive complex 2 (PRC2) in maintenance of this disease. shRNA-mediated suppression of PRC2 subunits Eed, Suz12 or Ezh1/Ezh2 led to proliferation arrest and differentiation of leukemia cells, with a minimal impact on growth of several non-transformed hematopoietic cell lines. The requirement for PRC2 in leukemia is partly because of its role in direct transcriptional repression of genes that limit the self-renewal potential of hematopoietic cells, including Cdkn2a. In addition to implicating a role for PRC2 in the pathogenesis of MLL-fusion leukemia, our results suggest, more generally, that Trithorax and Polycomb group proteins can cooperate with one another to maintain aberrant lineage programs in cancer.

Publication types

  • Research Support, N.I.H., Intramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Substitution
  • Animals
  • Aspartic Acid / genetics
  • Cell Proliferation*
  • Disease Models, Animal
  • Genes, ras / genetics*
  • Glycine / genetics
  • Humans
  • Leukemia, Myeloid, Acute / genetics*
  • Leukemia, Myeloid, Acute / metabolism
  • Leukemia, Myeloid, Acute / pathology
  • Mice
  • Mice, Transgenic
  • Models, Biological
  • Mutation, Missense / physiology
  • Oncogene Proteins, Fusion / genetics*
  • Polycomb Repressive Complex 2 / genetics
  • Polycomb Repressive Complex 2 / metabolism
  • Polycomb Repressive Complex 2 / physiology*
  • Polycomb-Group Proteins / genetics
  • Polycomb-Group Proteins / physiology
  • Tumor Cells, Cultured

Substances

  • MLL-AF9 fusion protein, mouse
  • Oncogene Proteins, Fusion
  • Polycomb-Group Proteins
  • Suz12 protein, mouse
  • Aspartic Acid
  • Polycomb Repressive Complex 2
  • Glycine