Strain differences and the genetic basis of experimental autoimmune anti-glomerular basement membrane glomerulonephritis

Authors

  • John Reynolds

    1. Division of Science, Faculty of Creative Arts, Technologies and Science, Park Square Campus, University of Bedfordshire, Luton, UK
    2. Renal Section, Division of Medicine, Hammersmith Campus, Imperial College London, UK
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John Reynolds
Division of Science
Faculty of Creative Arts
Technologies and Science
Park Square Campus
University of Bedfordshire
Luton, LU1 3JU
UK
Tel.: +44 (0) 158 2489299
Fax: +44 (0) 158 2489212
E-mail: john.reynolds@beds.ac.uk

Summary

Goodpasture’s, or anti-glomerular basement membrane (GBM), disease presents with rapidly progressive glomerulonephritis, caused by autoimmunity to a component of the GBM, the non-collagenous domain of the α3 chain of type IV collagen [α3(IV)NC1]. To investigate the mechanisms of inflammation in glomerulonephritis and to test new approaches to treatment, animal models of glomerulonephritis, termed experimental autoimmune glomerulonephritis (EAG), have been developed in susceptible strains of rats and mice. This review article describes how these models of EAG have been developed over the past three decades, discusses the evidence for the involvement of both humoral and cell-mediated immunity in the induction and pathogenesis of glomerulonephritis in these models and highlights recent, emerging data that have identified potential candidate genes that may control the genetic susceptibility in these different strains of rats and mice. The identification of these susceptibility genes has lead to a better understanding of the genetic basis of this model of anti-GBM disease, which may be relevant to the immunopathogenesis of Goodpasture’s disease, and more generally to the progression from autoimmunity to target-organ damage.

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