An MRL/MpJ-lpr/lpr substrain with a limited expansion of lpr double-negative T cells and a reduced autoimmune syndrome

The autosomal recessive mutant gene, lpr, 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-lpr/lpr (MRL-lpr) mice whic...

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Bibliographic Details
Published in:International immunology 1993-05, Vol.5 (5), p.525-532
Main Authors: Fossati, Liliane, Takahashi, Satoru, Merino, Ramón, Iwamoto, Masahiro, Aubry, Jean-Pierre, Nose, Masato, Spach, Colette, Motta, Roland, Izui, Shozo
Format: Article
Language:English
Subjects:
Animals
Autoantibodies - biosynthesis
Autoimmune Diseases - genetics
autoimmunity
Cryoglobulins - biosynthesis
Female
Genes, Recessive
Glomerulonephritis - genetics
Glomerulonephritis - immunology
Glomerulonephritis - pathology
In Vitro Techniques
Longevity - genetics
Lymphocyte Activation
lymphoproliferation
Male
Mice
Mice, Mutant Strains - genetics
Mice, Mutant Strains - immunology
mutant mouse
Species Specificity
Syndrome
systemic lupus erythematosus
T-Lymphocyte Subsets - immunology
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Summary:The autosomal recessive mutant gene, lpr, 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-lpr/lpr (MRL-lpr) mice which live almost twice as long with delayed development of glomerulonephritis, compared with conventional MRL-lpr mice. This substrain, termed MRL-lpr.II (II for long-lived), develops generalized lymphadenopathy characteristically seen in MRL-lpr mice. However, the expansion of a double negative lpr T cell subset is markedly limited with a 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 significantly differ between the two MRL-lpr mice. However, serum levels of cryoglobulins, whose major component is lgG3, are markedly diminished in MRL-lpr.ll mice with a parallel decrease in lgG3. Since MRL-lpr.ll mice still carry the lpr 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 pathogenesls of lupus-like autoimmune syndrome.
ISSN:0953-8178
1460-2377
DOI:10.1093/intimm/5.5.525