Genetic basis of the complex pathological manifestations of collagen disease: Lessons from MRL/lpr and related mouse models

Masato Nose, M. Nishihara, H. Fujii

Research output: Contribution to journalArticlepeer-review

10 Citations (Scopus)

Abstract

The pathological findings in collagen disease including systemic lupus erythematosus show complex lesions such as glomerulonephritis, systemic vasculitis, polyarthritis, sialoadenitis, etc. Moreover, some cases of collagen disease are categorized into overlapping syndromes. It is still controversial whether such diversity and similarity of pathological manifestations among the collagen disease depends on ambiguity in diagnosis or is an intrinsic quality of the collagen diseases themselves. In this paper, we reviewed this subject focusing on a series of our genetic studies of murine models of collagen disease, MRL strains of mice with a deficit in Fas-mediated apoptosis, which spontaneously develop glomerulonephritis, systemic vasculitis, polyarthritis and sialoadenitis. We observed that each lesion was controlled by a different set of genes and they appeared to act in an additive manner on the development of each lesion. We conclude that various disease categories in collagen disease will be a result of the combination of polygenes.

Original languageEnglish
Pages (from-to)473-498
Number of pages26
JournalInternational Reviews of Immunology
Volume19
Issue number4-5
DOIs
Publication statusPublished - 2000 Jan 1
Externally publishedYes

Keywords

  • Allelic polymorphism
  • Autoimmune disease
  • Fas
  • Genetic dissection
  • Polygene

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

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