Aberrant expression of Notch1 interferes with the B-lymphoid phenotype of neoplastic B cells in classical Hodgkin lymphoma

Leukemia. 2008 Aug;22(8):1587-94. doi: 10.1038/leu.2008.101. Epub 2008 May 1.

Abstract

Plasticity of committed mouse B cells has been demonstrated by inactivation of the B-cell commitment transcription factor PAX5, resulting in loss of the B-cell phenotype and differentiation into various hematopoietic lineages. Furthermore, mature mouse B cells could be reprogrammed into macrophages by overexpression of myeloid-specific transcription factors. Here, we report that aberrant activity of the transmembrane receptor, Notch1, interferes with the B-lymphoid phenotype of mature human germinal center-derived B cells in Hodgkin lymphoma, so called Hodgkin and Reed-Sternberg cells. They have lost the B-cell phenotype despite their mature B-cell origin. Notch1 remodels the B-cell transcription factor network by antagonizing the key transcription factors E2A and early B-cell factor (EBF). Through this mechanism, B lineage-specific genes were suppressed and B lineage-inappropriate genes were induced. We provide evidence that absence of the Notch inhibitor Deltex1 contributes to deregulated Notch activity in Hodgkin and Reed-Sternberg cells. These data suggest that Notch activation interferes with dedifferentiation of neoplastic B cells in Hodgkin lymphoma.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • B-Lymphocytes / cytology
  • B-Lymphocytes / immunology*
  • Base Sequence
  • Cell Differentiation
  • Cell Line, Tumor
  • Cell Lineage
  • DNA Primers
  • Hodgkin Disease / immunology
  • Hodgkin Disease / metabolism
  • Hodgkin Disease / pathology*
  • Humans
  • Immunohistochemistry
  • In Situ Hybridization
  • PAX5 Transcription Factor / metabolism
  • Phenotype
  • Receptor, Notch1 / metabolism*
  • Reverse Transcriptase Polymerase Chain Reaction

Substances

  • DNA Primers
  • PAX5 Transcription Factor
  • PAX5 protein, human
  • Receptor, Notch1