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CD3 ζ defects in systemic lupus erythematosus
  1. Tsutomu Takeuchi1,
  2. Katsuya Suzuki1,
  3. Tsuneo Kondo2,
  4. Keiko Yoshimoto1,
  5. Kensei Tsuzaka3
  1. 1Department of Internal Medicine, Division of Rheumatology, School of Medicine, Keio University, Tokyo, Japan
  2. 2Department of Rheumatology/Clinical Immunology, Saitama Medical Center, Saitama Medical University, Tokyo, Japan
  3. 3Department of Internal Medicine, Ichikawa General Hospital, Tokyo Dental College, Tokyo, Japan
  1. Correspondence to Professor Tsutomu Takeuchi, 35 Shinanomachi, Shinjyuku, Tokyo 160–8552, Japan; tsutake{at}


The prototype autoimmune disease, systemic lupus erythematosus (SLE), has been known to be associated with deficiency of ζ chain, a component of the T-cell receptor–CD3 complex. Comprehensive analysis has shown that expression of the CD3 ζ chain is attenuated or absent in over half of SLE patients. Furthermore, aberrant transcripts of the CD3 ζ 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 ζ chain is also observed in cancer patients, infections and other autoimmune diseases, sustained attenuation of the CD3 ζ expression accompanied with aberrant transcripts are only observed in SLE. In this study, the authors review the unique features of CD3 ζ 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.

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  • Competing interests None.

  • Provenance and peer review Commissioned; externally peer reviewed.

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