Autoimmune Disease Spontaneous Animal Models

Georg Wick and Roswitha Sgonc, Institute for General and Experimental Pathology, University of Innsbruck, Innsbruck, Austria

Guido Kroemer, Centre National de la Recherche Scientifique - UPR 420, Villejuif, France

Autoimmune diseases can be experimentally induced by immunization of normal animals with the respective antigens combined with appropriate adjuvants. By this means organ-specific experimental autoimmune diseases can be produced in animals of many different strains and in nearly every organ. There is no doubt that such experimentally induced autoimmune diseases, e.g. experimental allergic encephalomyelitis (EAE) or experimental autoimmune thyroiditis (EAT), have contributed considerably to our understanding of the pathogenesis of autoimmune diseases in general and these models in particular. It is, however, also clear that most of these artificially produced autoimmune diseases often do not parallel their human counterparts with respect to all essential characteristics.

Fortunately, there are several animal strains available that - based on many years of selective breeding - develop certain organ-specific or systemic autoimmune diseases spontaneously, i.e. without any experimental manipulation. These models mimic the human situation much more closely and thus serve as apt objects of study for the pathogenetic mechanisms underlying the development of these diseases as well as the establishment of new diagnostic and therapeutic strategies. In this entry, spontaneous animal models for four types of diseases, two systemic and two organ specific, will be presented. This selection is based on the large body of literature on these models, but it should be emphasized that several other promising strains exist which have not yet been investigated in such depth but may also turn out to be very useful in the future, such as the Buffalo (BUF) rat that develops an autoimmune thyroiditis in high frequency, the autoimmune glomerulonephritis, arthritis and orchitis emerging in Aleutian minks, the delayed amenalosis (DAM) chicken with autoimmune depigmentation, and several others.

In principle, spontaneous autoimmune models arose due to a combination of serendipity (spontaneous mutations causing autoimmune phenotypes) and inbreeding plus selection procedures. As a consequence, inbred animal strains afflicted by spontaneous autoimmune diseases usually manifest disease phenotypes that are compatible with breeding programs, i.e. that do not cause severe pathology before animals reach sexual maturity, although they sometimes may need special dietary, hormonal, etc. supplementation. Alternatively, autoimmune phenotypes are studied in severely diseased F1 crosses of relatively normal parental inbred strains. This particular feature of spontaneous autoimmune disease models may explain why certain laboratory-produced mutations (e.g. disruption of the CTLA4 gene) that give rise to an acute, fulminant autoimmune disorder have not been observed spontaneously.

Arthritis Relief Now

Arthritis Relief Now

When you hear the word arthritis, images of painful hands and joints comes into play. Few people fully understand arthritis and this guide is dedicated to anyone suffering with this chronic condition and wants relief now.

Get My Free Ebook


Post a comment