Haploinsufficiency of Bcl11b for suppression of lymphomagenesis and thymocyte development

Biochem Biophys Res Commun. 2007 Apr 6;355(2):538-42. doi: 10.1016/j.bbrc.2007.02.003. Epub 2007 Feb 8.

Abstract

Recurrent chromosomal rearrangements at BCL11B are found in human hematopoietic malignancies mostly of T-cell origin. However, it is unclear how this disruption contributes to oncogenesis, because the majority of leukemias express BCL11B from an undisrupted allele. Here, we show that Bcl11b(+/-)p53(+/-) mice exhibited greater susceptibility to lymphomas than Bcl11b(+/+)p53(+/-) mice but most lymphomas retained and expressed the wild-type Bcl11b allele. This strongly suggests that Bcl11b is haploinsufficient for suppression of thymic lymphoma development in mice of the p53(+/-) background, a situation in which functional loss of only one allele confers a selective advantage for tumor growth. The haploinsufficiency is further supported by that Bcl11b(+/-) mouse embryos were impaired in thymocyte development and survival. These results indicate relevance of BCL11B aberration to human leukemogenesis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Base Sequence
  • Cell Differentiation
  • DNA Primers
  • DNA-Binding Proteins / genetics*
  • DNA-Binding Proteins / immunology
  • Flow Cytometry
  • Gamma Rays
  • Genes, Tumor Suppressor
  • Genetic Predisposition to Disease
  • Genotype
  • Haplotypes
  • Lymphoma / genetics
  • Mice
  • Mice, Inbred BALB C
  • Repressor Proteins / genetics*
  • Repressor Proteins / immunology
  • Thymus Gland / cytology
  • Thymus Neoplasms / genetics
  • Tumor Suppressor Proteins / genetics*
  • Tumor Suppressor Proteins / immunology

Substances

  • Bcl11b protein, mouse
  • DNA Primers
  • DNA-Binding Proteins
  • Repressor Proteins
  • Tumor Suppressor Proteins