The Theory of APL

Oncogene. 2001 Oct 29;20(49):7216-22. doi: 10.1038/sj.onc.1204855.

Abstract

Acute promyelocytic leukemia (APL) is associated with reciprocal and balanced chromosomal translocations always involving the Retinoic Acid Receptor alpha (RARalpha) gene on chromosome 17 and variable partner genes (X genes) on distinct chromosomes. RARalpha fuses to the PML gene in the vast majority of APL cases, and in a few cases to the PLZF, NPM, NuMA and STAT5b genes. As a consequence, X-RARalpha and RARalpha-X fusion genes are generated encoding aberrant fusion proteins that can interfere with X and/or RARalpha function. Here we will review the relevant conclusions and the open questions that stem from a decade of in vivo analysis of APL pathogenesis in the mouse in transgenic and knock-out models.

Publication types

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

MeSH terms

  • Animals
  • Cell Transformation, Neoplastic / genetics
  • Disease Models, Animal
  • Humans
  • Leukemia, Promyelocytic, Acute / genetics*
  • Mice
  • Mice, Knockout
  • Mice, Transgenic
  • Oncogene Proteins, Fusion / genetics*
  • Receptors, Retinoic Acid / genetics*
  • Retinoic Acid Receptor alpha
  • Translocation, Genetic*

Substances

  • Oncogene Proteins, Fusion
  • RARA protein, human
  • Rara protein, mouse
  • Receptors, Retinoic Acid
  • Retinoic Acid Receptor alpha