Breakdown of Immune Tolerance in AIRE-Deficient Rats Induces a Severe Autoimmune Polyendocrinopathy–Candidiasis–Ectodermal Dystrophy–Like Autoimmune Disease

    June 2018 in “ ˜The œjournal of immunology/˜The œJournal of immunology ”
    Jason Ossart, Anne Moreau, Elodie Autrusseau, Séverine Ménoret, Jérôme C. Martin, Marine Besnard, Laure‐Hélène Ouisse, Laurent Tesson, Léa Flippe, Kai Kisand, Pärt Peterson, François‐Xavier Hubert, Ignacio Anegón, Régis Josien, Carole Guillonneau
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    TLDR AIRE-deficient rats developed severe autoimmune disease similar to APECED, useful for testing treatments.
    Researchers created the first AIRE-deficient rat model using zinc-finger nucleases to study autoimmune polyendocrinopathy–candidiasis–ectodermal dystrophy (APECED). This model displayed key APECED symptoms such as alopecia, skin depigmentation, and nail dystrophy, and showed severe autoimmune lesions in various organs, particularly the pancreas. The rats also had autoantibodies and cytokine levels similar to those in APECED patients. This model provided a relevant tool for testing potential curative treatments for APECED.
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