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

First Author  Nose M Year  2000
Journal  Int Rev Immunol Volume  19
Issue  4-5 Pages  473-98
PubMed ID  11016428 Mgi Jnum  J:66638
Mgi Id  MGI:1929987 Doi  10.3109/08830180009055508
Citation  Nose M, et al. (2000) Genetic basis of the complex pathological manifestations of collagen disease: lessons from MRL/lpr and related mouse models. Int Rev Immunol 19(4-5):473-98
abstractText  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.
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