An MRL/MpJ-lpr/lpr substrain with a limited expansion of lpr double-negative T cells and a reduced autoimmune syndrome |
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Authors: | Fossati Liliane; Takahashi Satoru; Merino Ramon; Iwamoto Masahiro; Aubry Jean-Pierre; Nose Masato; Spach Colette; Motta Roland; Izui Shozo |
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Institution: | Department of Pathology, Centre Médical Universitaire, University of Geneva Geneva Switzerland
1 Glaxo Institute for Molecular Biology Geneva Switzerland
2 Department of Pathology, Tohoku University School of Medicine Sendai Japan
3 Laboratoire d'lmmunogénétique, CNRS, Centre Marcel Delépine Orléans, France |
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Abstract: | The autosomal recessive mutant gene, lpr, has been shown toaccelerate the progression of lupus-like autoimmune disease,which is associated with a massive expansion of a unique CD4–CD8–double-negative T cell subset, in MRL/MpJ mice. Here we reporta substrain of MRL/MpJ-lpr/lpr (MRL-lpr) mice which live almosttwice as long with delayed development of glomerulonephritis,compared with conventional MRL-lpr mice. This substrain, termedMRL-lpr.II (II for long-lived), develops generalized lymphadenopathycharacteristically seen in MRL-lpr mice. However, the expansionof a double negative lpr T cell subset is markedly limited witha mean value of 15% in their lymph nodes compared to about 70%in conventional MRL-lpr mice. Overall production of autoantibodies,such as anti-DNA and rheumatoid factors, does not significantlydiffer between the two MRL-lpr mice. However, serum levels ofcryoglobulins, whose major component is lgG3, are markedly diminishedin MRL-lpr.ll mice with a parallel decrease in lgG3. Since MRL-lpr.llmice still carry the lpr mutation, as documented by the presenceof defects in the Fas antigen, a possible new mutation in thissubstrain may play a significant role in the pathogenesls oflupus-like autoimmune syndrome. |
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Keywords: | autoimmunity lymphoproliferation mutant mouse systemic lupus erythematosus |
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