An mrl/mpj-/pr//pr substrain with a limited expansion of ipr double-negative t cells and a reduced autoimmune syndrome

Liliane Fossati, Satoru Takahashi, Ramon Merino, Masahiro Iwamoto, Jean Pierre Aubry, Masato Nose, Colette Spach, Roland Motta, Shozo Izui

Research output: Contribution to journalArticlepeer-review

34 Citations (Scopus)

Abstract

The autosomal recessive mutant gene, Ipr, has been shown to accelerate 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 report a substrain of MRL/MpJ-fpr//pr (MRL-fpr) mice which live almost twice as long with delayed development of glomerulonephritis, compared with conventional MRL-/pr mice. This substrain, termed MRL-/pr.//(//for long-lived), develops generalized lymphadenopathy characteristically seen in MRL-/pr mice. However, the expansion of a double negative Ipr T cell subset is markedly limited with a mean value of 15% in their lymph nodes compared to about 70% in conventional MRL-/pr mice. Overall production of autoantibodies, such as anti-DNA and rheumatoid factors, does not significantly differ between the two MRL-/pr mice. However, serum levels of cryoglobulins, whose major component is lgG3, are markedly diminished in MRL-/pr.//mice with a parallel decrease in lgG3. Since MRL-/pr.//mice still carry the Ipr mutation, as documented by the presence of defects in the Fas antigen, a possible new mutation in this substrain may play a significant role in the pathogenesis of lupus-like autoimmune syndrome.

Original languageEnglish
Pages (from-to)525-532
Number of pages8
JournalInternational immunology
Volume5
Issue number5
DOIs
Publication statusPublished - 1993 May

Keywords

  • Autoimmunity
  • Lymphoproliferation
  • Mutant mouse
  • Systemic lupus erythematosus

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

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