Apoptosis-stimulating protein of p53 (ASPP2) heterozygous mice are tumor-prone and have attenuated cellular damage-response thresholds

Proc Natl Acad Sci U S A. 2009 Mar 17;106(11):4390-5. doi: 10.1073/pnas.0809080106. Epub 2009 Feb 26.

Abstract

The expression of ASPP2 (53BP2L), a proapoptotic member of a family of p53-binding proteins, is frequently suppressed in many human cancers. Accumulating evidence suggests that ASPP2 inhibits tumor growth; however, the mechanisms by which ASPP2 suppresses tumor formation remain to be clarified. To study this, we targeted the ASPP2 allele in a mouse by replacing exons 10-17 with a neoR gene. ASPP2(-/-) mice were not viable because of an early embryonic lethal event. Although ASPP2(+/-) mice appeared developmentally normal, they displayed an increased incidence of a variety of spontaneous tumors as they aged. Moreover, gamma-irradiated 6-week-old ASPP2(+/-) mice developed an increased incidence of high-grade T cell lymphomas of thymic origin compared with ASPP2(+/+) mice. Primary thymocytes derived from ASPP2(+/-) mice exhibited an attenuated apoptotic response to gamma-irradiation compared with ASPP2(+/+) thymocytes. Additionally, ASPP2(+/-) primary mouse embryonic fibroblasts demonstrated a defective G(0)/G(1) cell cycle checkpoint after gamma-irradiation. Our results demonstrate that ASPP2 is a haploinsufficient tumor suppressor and, importantly, open new avenues for investigation into the mechanisms by which disruption of ASPP2 pathways could play a role in tumorigenesis and response to therapy.

Publication types

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

MeSH terms

  • Animals
  • Apoptosis / radiation effects
  • Apoptosis Regulatory Proteins / genetics*
  • Cell Cycle / radiation effects
  • Gamma Rays
  • Genetic Predisposition to Disease
  • Heterozygote
  • Lymphoma, T-Cell / etiology
  • Lymphoma, T-Cell / genetics
  • Mice
  • Mice, Mutant Strains
  • Neoplasms / etiology
  • Neoplasms / genetics
  • Thymus Gland
  • Tumor Suppressor Proteins / genetics*

Substances

  • Apoptosis Regulatory Proteins
  • Trp53bp2 protein, mouse
  • Tumor Suppressor Proteins