Increased frequency of RAG-expressing, CD4+CD3(low) peripheral T lymphocytes in patients with defective responses to DNA damage

Erica Lantelme, Stefania Mantovani, Belinda Palermo, Rita Campanelli, Luisa Granziero, Virginia Monafo, Claudia Giachino

Research output: Contribution to journalArticlepeer-review


Accumulating evidence indicates that peripheral lymphocyte variants with altered antigen receptor expression may be capable of expressing recombination-activating genes (RAG). We and others recently observed functional RAG gene products in mature T cells with defective TCR expression. Here, the association between TCR expression and RAG activity was assessed further in lymphocytes from patients with defective responses to DNA damage. We show that T cells with altered TCR surface expression are present in increased numbers in these patients and that they express RAG genes. The finding of RAG gene expression by TCR variants suggests the possibility that secondary V(D)J rearrangements could be induced in these cells to rescue their defective phenotype and cellular function. Moreover, as V(D)J recombination has been implicated in chromosome translocations involving antigen receptor genes, we discuss a possible relationship between altered TCR expression, RAG activity and the frequent lymphoma-specific translocations observed in these patients.

Original languageEnglish
Pages (from-to)1520-1525
Number of pages6
JournalEuropean Journal of Immunology
Issue number5
Publication statusPublished - 2000


  • Antigen receptor
  • DNA repair
  • Human
  • Recombination
  • Somatic mutation

ASJC Scopus subject areas

  • Immunology


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