A novel macromolecular structure is a target of the promyelocyte-retinoic acid receptor oncoprotein

Cell. 1994 Jan 28;76(2):333-43. doi: 10.1016/0092-8674(94)90340-9.

Abstract

Acute promyelocytic leukemia (APL) is associated with a t(15;17) translocation that creates the promyelocyte-retinoic acid receptor alpha (PML-RAR alpha) fusion gene. Immunohistochemistry demonstrates that PML is a part of a novel macromolecular organelle (including at least three other nuclear proteins) referred to as PML oncogenic domains (PODs). In APL cells, the POD is disrupted into a microparticulate pattern as a consequence of the expression of the PML-RAR oncoprotein. RA treatment of APL cells triggers a reorganization of PML to generate normal-appearing PODs. We propose that PML-RAR is a dominant negative oncoprotein that exerts its putative leukomogenic effect by inhibiting assembly of the POD. According to this proposal, not only is the POD a novel structure, but it can be ascribed an imputed function such that its disruption leads to altered myeloid maturation; this may represent a novel oncogenic target.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Cell Nucleus / ultrastructure*
  • Chromosomes, Human, Pair 15
  • Chromosomes, Human, Pair 17
  • Fluorescent Antibody Technique
  • Humans
  • Leukemia, Promyelocytic, Acute / pathology*
  • Macromolecular Substances
  • Neoplasm Proteins*
  • Nuclear Proteins / metabolism
  • Oncogenes
  • Promyelocytic Leukemia Protein
  • Receptors, Retinoic Acid / immunology
  • Receptors, Retinoic Acid / metabolism*
  • Transcription Factors / immunology
  • Transcription Factors / metabolism*
  • Translocation, Genetic
  • Tretinoin / pharmacology
  • Tumor Suppressor Proteins

Substances

  • Macromolecular Substances
  • Neoplasm Proteins
  • Nuclear Proteins
  • Promyelocytic Leukemia Protein
  • Receptors, Retinoic Acid
  • Transcription Factors
  • Tumor Suppressor Proteins
  • PML protein, human
  • Tretinoin