Correction of accelerated autoimmune disease by early replacement of the mutated lpr gene with the normal Fas apoptosis gene in the T cells of transgenic MRL-lpr/lpr mice

Proc Natl Acad Sci U S A. 1994 Mar 15;91(6):2344-8. doi: 10.1073/pnas.91.6.2344.

Abstract

MRL-lpr/lpr mice develop a generalized autoimmune disease which includes increased autoantibody production, glomerulonephritis, and development of lymphadenopathy. The lpr genetic defect has been identified as a mutation in the Fas apoptosis gene that results in low expression of Fas mRNA. To determine the significance of the lpr mutation and T cells in the development of the autoimmune disease, we constructed transgenic MRL-lpr/lpr mice using a full-length murine Fas cDNA under the regulation of the T-cell-specific CD2 promoter and enhancer. Here we show that the early correction of the lpr gene defect in T cells eliminates glomerulonephritis and development of lymphadenopathy and decreases the levels of autoantibodies. In this model, early correction of the lpr defect in T cells is sufficient to eliminate the acceleration of autoimmune disease even in the presence of B cells and other cells that express the mutant lpr gene.

Publication types

  • Research Support, U.S. Gov't, Non-P.H.S.
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Antigens, Surface / genetics*
  • Apoptosis / genetics*
  • Autoimmune Diseases / genetics*
  • Autoimmune Diseases / therapy
  • Base Sequence
  • Blotting, Northern
  • DNA
  • Enhancer Elements, Genetic
  • Fluorescent Antibody Technique
  • Humans
  • Lymphoproliferative Disorders / genetics*
  • Lymphoproliferative Disorders / immunology
  • Mice
  • Mice, Transgenic
  • Molecular Sequence Data
  • Mutation
  • Promoter Regions, Genetic
  • T-Lymphocytes / cytology*
  • fas Receptor

Substances

  • Antigens, Surface
  • fas Receptor
  • DNA