The nonhomologous end joining factor Artemis suppresses multi-tissue tumor formation and prevents loss of heterozygosity

Oncogene. 2007 Sep 6;26(41):6010-20. doi: 10.1038/sj.onc.1210430. Epub 2007 Mar 26.

Abstract

Nonhomologous end joining (NHEJ) is a critical DNA repair pathway, with proposed tumor suppression functions in many tissues. Mutations in the NHEJ factor ARTEMIS cause radiation-sensitive severe combined immunodeficiency in humans and may increase susceptibility to lymphoma in some settings. We now report that deficiency for Artemis (encoded by Dclre1c/Art in mouse) accelerates tumorigenesis in several tissues in a Trp53 heterozygous setting, revealing tumor suppression roles for NHEJ in lymphoid and non-lymphoid cells. We also show that B-lineage lymphomas in these mice undergo loss of Trp53 heterozygosity by allele replacement, but arise by mechanisms distinct from those in Art Trp53 double null mice. These findings demonstrate a general tumor suppression function for NHEJ, and reveal that interplay between NHEJ and Trp53 loss of heterozygosity influences the sequence of multi-hit oncogenesis. We present a model where p53 status at the time of tumor initiation is a key determinant of subsequent oncogenic mechanisms. Because Art deficient mice represent a model for radiation-sensitive severe combined immunodeficiency, our findings suggest that these patients may be at risk for both lymphoid and non-lymphoid cancers.

Publication types

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

MeSH terms

  • Animals
  • DNA Repair*
  • Endonucleases
  • Genes, p53*
  • Humans
  • Loss of Heterozygosity*
  • Lymphoma / genetics
  • Lymphoma / pathology
  • Mice
  • Mice, Knockout
  • Neoplasms / genetics*
  • Neoplasms / pathology*
  • Nuclear Proteins / deficiency
  • Nuclear Proteins / genetics*
  • Sarcoma, Experimental / genetics
  • Sarcoma, Experimental / pathology
  • Severe Combined Immunodeficiency / genetics
  • Tumor Suppressor Protein p53 / deficiency

Substances

  • Nuclear Proteins
  • Tumor Suppressor Protein p53
  • Endonucleases
  • Dclre1c protein, mouse