Prkar1a is an osteosarcoma tumor suppressor that defines a molecular subclass in mice

J Clin Invest. 2010 Sep;120(9):3310-25. doi: 10.1172/JCI42391. Epub 2010 Aug 9.

Abstract

Some cancers have been stratified into subclasses based on their unique involvement of specific signaling pathways. The mapping of human cancer genomes is revealing a vast number of somatic alterations; however, the identification of clinically relevant molecular tumor subclasses and their respective driver genes presents challenges. This information is key to developing more targeted and personalized cancer therapies. Here, we generate a new mouse model of genomically unstable osteosarcoma (OSA) that phenocopies the human disease. Integrative oncogenomics pinpointed cAMP-dependent protein kinase type I, alpha regulatory subunit (Prkar1a) gene deletions at 11qE1 as a recurrent genetic trait for a molecularly distinct subclass of mouse OSA featuring RANKL overexpression. Using mouse genetics, we established that Prkar1a is a bone tumor suppressor gene capable of directing subclass development and driving RANKL overexpression during OSA tumorigenesis. Finally, we uncovered evidence for a PRKAR1A-low subset of human OSA with distinct clinical behavior. Thus, tumor subclasses develop in mice and can potentially provide information toward the molecular stratification of human cancers.

Publication types

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

MeSH terms

  • Animals
  • Cyclic AMP-Dependent Protein Kinase RIalpha Subunit / genetics*
  • Disease Models, Animal
  • Gene Deletion
  • Genes, Tumor Suppressor*
  • Mice
  • Mice, Transgenic
  • Osteosarcoma / genetics*
  • Phenotype

Substances

  • Cyclic AMP-Dependent Protein Kinase RIalpha Subunit
  • Prkar1a protein, mouse