Mutations in the IkBa gene in Hodgkin's disease suggest a tumour suppressor role for IκBα

Eric Cabannes, Gulfaraz Khan, Fabienne Aillet, Ruth F. Jarrett, Ronald T. Hay

Research output: Contribution to journalArticlepeer-review

291 Citations (Scopus)


The NF-κB/Rel family of transcription factors regulates a wide variety of genes whose products play a fundamental role in inflammatory and immune responses. The implication of NF-κB/Rel proteins and their IκB regulatory subunits in the control of cellular growth and oncogenesis, was suggested by the induction of fatal lymphomas in birds by the v-rel oncoprotein, and the rearrangement and amplification of several genes encoding the NF-κB/Rel/IκB signal transduction factors in human malignancies, primarily of lymphoid origin. Hodgkin's disease (HD) is a lymphoma characterized by a low frequency of malignant Hodgkin and Reed-Sternberg (H/RS) cells in a reactive background of nonneoplastic cells. The peculiar activated phenotype of Hodgkin and Reed-Sternberg cells and their pattern of cytokine secretion are believed to be a consequence of constitutive activation of the NF-κB transcription factor. Here, we report the detection of mutations of the IκBa gene, in two HD-derived cell lines and in two out of eight biopsy samples from patients with relapsed Hodgkin's disease. The presence of defective IκBα is thus likely to explain the constitutive activation of NF-κB in these cells and suggests that IκBα is a tumour suppressor controlling the oncogenic activation of NF-κB in Hodgkin and Reed-Sternberg cells.

Original languageEnglish
Pages (from-to)3063-3070
Number of pages8
Issue number20
Publication statusPublished - May 20 1999
Externally publishedYes


  • Hodgkin's lymphoma
  • IκBα
  • NF-κB
  • Tumour suppressor

ASJC Scopus subject areas

  • Molecular Biology
  • Genetics
  • Cancer Research


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