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Abstract
IgA nephropathy (IgAN) is the most common primary glomerulonephritis in the world. It was first described in 1968 by Jean Berger and Nicole Hinglais as the presence of intercapillary deposits of IgA. Despite this simple description, patients with IgAN may present very broad clinical features ranging from the isolated presence of IgA in the mesangium without clinical or biological manifestations to rapidly progressive kidney failure. These features are associated with a variety of histological lesions, from the discrete thickening of the mesangial matrix to diffuse cell proliferation. Immunofluorescence on IgAN kidney specimens shows the isolated presence of IgA or its inconsistent association with IgG and complement components. This clinical heterogeneity of IgAN clearly echoes its complex and multifactorial pathophysiology in humans, inviting further analyses of its various aspects through the use of experimental models. Small-animal models of IgAN provide the most pertinent strategies for studying the multifactorial aspects of IgAN pathogenesis and progression. Although only primates have the IgA1 subclass, several murine models have been developed in which various aspects of immune responses are deregulated and which are useful in the understanding of IgAN physiopathology as well as in the assessment of IgAN therapeutic approaches. In this manuscript, we review all murine IgAN models developed since 1968 and discuss their remarkable contribution to understanding the disease.
Highlights
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Abnormalities in the microbiota following a modification in the environment or an alteration of the immune response and of the repertoire can promote the development of an immunopathological process; IgA nephropathy (IgAN) could be such an example
Using a transgenic human IgA-1 producing model lacking the DNA-editing enzyme activation induced cytidine deaminase (AID), responsible for IgA affinity maturation, we showed that IgA deposition and complement activation significantly increased and led to IgAN pathogenesis, without significant proteinuria and hematuria
Summary
The true prevalence of IgAN cannot be exactly determined. IgA deposits on the kidney are frequent in asymptomatic patients and were reported in 16.1% of a population of kidney donors in Japan [1]. The immune response to the presence of various commensal microorganisms constitute a homeostatic immune repertoire that echoes the variability of the microbiota which evolves according to age, environment, and diet and which is accompanied by an increase in the IgA affinity, whether T-dependent or not. This repertoire is not limited to controlling the commensal microbiota, it is the source of protective immunity against pathogens [8,10]. Abnormalities in the microbiota following a modification in the environment or an alteration of the immune response and of the repertoire can promote the development of an immunopathological process; IgAN could be such an example
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