Abrogation of lupus nephritis in activation-induced deaminase-deficient MRL/lpr mice

J Immunol. 2007 Jun 1;178(11):7422-31. doi: 10.4049/jimmunol.178.11.7422.

Abstract

We generated MRL/lpr mice deficient in activation-induced deaminase (AID). Because AID is required for Ig hypermutation and class switch recombination, these mice lack hypermutated IgG Abs. Unlike their AID wild-type littermates, AID-deficient MRL/lpr mice not only lacked autoreactive IgG Abs but also experienced a dramatic increase in the levels of autoreactive IgM. This phenotype in AID-deficient mice translated into a significant reduction in glomerulonephritis, minimal mononuclear cell infiltration in the kidney, and a dramatic increase in survival to levels comparable to those previously reported for MRL/lpr mice completely lacking B cells and well below those of mice lacking secreted Abs. Therefore, this study wherein littermates with either high levels of autoreactive IgM or autoreactive IgG were directly examined proves that autoreactive IgM Abs alone are not sufficient to promote kidney disease in MRL/lpr mice. In addition, the substantial decrease in mortality combined with a dramatic increase in autoreactive IgM Abs in AID-deficient MRL/lpr mice suggest that autoreactive IgM Abs might not only fail to promote nephritis but may also provide a protective role in MRL/lpr mice. This novel mouse model containing high levels of autoreactive, unmutated IgM Abs will help delineate the contribution of autoreactive IgM to autoimmunity.

Publication types

  • Comparative Study
  • Research Support, N.I.H., Intramural

MeSH terms

  • Animals
  • Autoantibodies / biosynthesis
  • Autoantibodies / metabolism
  • B-Lymphocyte Subsets / enzymology
  • B-Lymphocyte Subsets / immunology
  • B-Lymphocyte Subsets / pathology
  • Cell Movement / genetics
  • Cell Movement / immunology
  • Cytidine Deaminase / biosynthesis
  • Cytidine Deaminase / deficiency*
  • Cytidine Deaminase / genetics*
  • Immunoglobulin G / biosynthesis
  • Immunoglobulin G / metabolism
  • Immunoglobulin M / biosynthesis
  • Immunoglobulin M / metabolism
  • Kidney / enzymology
  • Kidney / pathology
  • Leukocytes, Mononuclear / enzymology
  • Leukocytes, Mononuclear / pathology
  • Lupus Nephritis / enzymology*
  • Lupus Nephritis / immunology
  • Lupus Nephritis / mortality
  • Lupus Nephritis / prevention & control*
  • Lymphocyte Count
  • Mice
  • Mice, Inbred BALB C
  • Mice, Inbred C57BL
  • Mice, Inbred MRL lpr
  • Mice, Knockout
  • Organ Specificity / genetics
  • Organ Specificity / immunology
  • Species Specificity
  • Survival Analysis
  • T-Lymphocyte Subsets / enzymology
  • T-Lymphocyte Subsets / immunology
  • T-Lymphocyte Subsets / pathology

Substances

  • Autoantibodies
  • Immunoglobulin G
  • Immunoglobulin M
  • AICDA (activation-induced cytidine deaminase)
  • Cytidine Deaminase