CD3 ξ defects in systemic lupus erythematosus

Tsutomu Takeuchi, Katsuya Suzuki, Tsuneo Kondo, Keiko Yoshimoto, Kensei Tsuzaka

Research output: Contribution to journalArticlepeer-review

23 Citations (Scopus)


The prototype autoimmune disease, systemic lupus erythematosus (SLE), has been known to be associated with deficiency of ξ chain, a component of the T-cell receptor-CD3 complex. Comprehensive analysis has shown that expression of the CD3 ξ chain is attenuated or absent in over half of SLE patients. Furthermore, aberrant transcripts of the CD3 ξ chain, including spliced variants lacking exon 7 or having a short 3′-untranslated region, have been detected in SLE T cells. Although attenuated expression of the CD3 ξ chain is also observed in cancer patients, infections and other autoimmune diseases, sustained attenuation of the CD3 ξ expression accompanied with aberrant transcripts are only observed in SLE. In this study, the authors review the unique features of CD3 ξ defects observed in SLE and discuss the molecular basis of the defects by recent findings in animal models, single-nucleotide polymorphisms and genome-wide association studies.

Original languageEnglish
Pages (from-to)i78-i81
JournalAnnals of the rheumatic diseases
Issue numberSUPPL. 2
Publication statusPublished - 2012 Apr

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology
  • Biochemistry, Genetics and Molecular Biology(all)


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