TY - JOUR
T1 - CD3 ξ defects in systemic lupus erythematosus
AU - Takeuchi, Tsutomu
AU - Suzuki, Katsuya
AU - Kondo, Tsuneo
AU - Yoshimoto, Keiko
AU - Tsuzaka, Kensei
PY - 2012/4
Y1 - 2012/4
N2 - 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.
AB - 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.
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U2 - 10.1136/annrheumdis-2011-200641
DO - 10.1136/annrheumdis-2011-200641
M3 - Article
C2 - 22460144
AN - SCOPUS:84859506368
VL - 71
SP - i78-i81
JO - Annals of the Rheumatic Diseases
JF - Annals of the Rheumatic Diseases
SN - 0003-4967
IS - SUPPL. 2
ER -