Mechanism for ubiquitylation of the leukemia fusion proteins AML1-ETO and PML-RARalpha

FASEB J. 2008 May;22(5):1369-79. doi: 10.1096/fj.06-8050com. Epub 2007 Dec 11.

Abstract

The chromosomal translocation products AML1-ETO and PML-RARalpha contribute to the pathogenesis of leukemias. Here, we demonstrate that both AML1-ETO and PML-RARalpha are degraded by the ubiquitin-proteasome system and that their turnover critically depends on the E2-conjugase UbcH8 and the E3-ligase SIAH-1. Contrary to its role in HDAC2 degradation, the E3-ligase RLIM does not target AML1-ETO and PML-RARalpha for ubiquitin-dependent elimination. RLIM rather is a substrate of SIAH-1, which indicates that these E3-ligases operate in a hierarchical order. Remarkably, proteasomal degradation of leukemia fusion proteins, in addition to the block of histone deacetylase (HDAC) enzymatic activity is a consequence of HDAC-inhibitor treatment. The former requires the induction of UbcH8 expression and each of these processes might be beneficial for leukemia treatment. Our observations shed light on the mechanism determining the interplay between E2-conjugases, E3-ligases, and their substrates and suggest a strategy for utilizing the ubiquitylation machinery in a therapeutic setting.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cell Line, Tumor
  • Core Binding Factor Alpha 2 Subunit / metabolism*
  • Histone Deacetylase Inhibitors
  • Humans
  • Nuclear Proteins / genetics
  • Nuclear Proteins / physiology
  • Oncogene Proteins, Fusion / metabolism*
  • RUNX1 Translocation Partner 1 Protein
  • Receptors, Retinoic Acid / metabolism
  • Ubiquitin-Conjugating Enzymes / physiology
  • Ubiquitin-Protein Ligases / genetics
  • Ubiquitin-Protein Ligases / physiology
  • Ubiquitination*
  • Valproic Acid / pharmacology

Substances

  • AML1-ETO fusion protein, human
  • Core Binding Factor Alpha 2 Subunit
  • Histone Deacetylase Inhibitors
  • Nuclear Proteins
  • Oncogene Proteins, Fusion
  • RUNX1 Translocation Partner 1 Protein
  • Receptors, Retinoic Acid
  • promyelocytic leukemia-retinoic acid receptor alpha fusion oncoprotein
  • Valproic Acid
  • UBE2L6 protein, human
  • Ubiquitin-Conjugating Enzymes
  • Ubiquitin-Protein Ligases
  • seven in absentia proteins