TCR ζ chain lacking exon 7 in two patients with systemic lupus erythematosus

Tsutomu Takeuchi, Kensei Tsuzaka, Ming Pang, Kouichi Amano, Jun Koide, Tohru Abe

研究成果: Article査読

66 被引用数 (Scopus)


To address the molecular mechanism underlying the functional defects of peripheral T cells in systemic lupus erythematosus (SLE), we focused on early signaling events. We demonstrated that protein expression of the TCR ζ chain was significantly decreased in peripheral T cells from patients with SLE compared to normal controls and patients with systemic sclerosis (SSc). Among those patients showing decreased TCR ζ chain expression, we found two patients with pronounced TCR ζ chain abnormalities, including an aberrant 14 kDa form in one and only trace expression in the other. RT-PCR, SSCP and subsequent cloning of the transcripts revealed that bases 468-503, corresponding to exon 7, were deleted in both patients. Since exon 7 spans the GTP/GDP binding site and N-terminal tyrosine in the third ITAM domain of TCR ζ chain, the transcript lacking exon 7 may be responsible for altered signal transduction via TCR in these SLE patients.

ジャーナルInternational immunology
出版ステータスPublished - 1998

ASJC Scopus subject areas

  • 免疫アレルギー学
  • 免疫学


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