AIH in a murine APS-1 model is directed against multiple autoantigens and can be controlled by polyspecific Tregs

Background and Aims: The autoimmune polyendocrine syndrome type 1 (APS-1) is caused by genetic aberrations of the autoimmune regulator (Aire) gene and manifests as spontaneous autoimmunity against multiple organs. We planned to investigate autoimmune hepatitis (AIH) in an APS-1 reflecting mouse model. Methods: In this APS-1 model, Aire at exon 2 was truncated (Aire-Δex2) on BALB/c background. We analyzed the pathophysiology of AIH, compared the impact of different Aire mutations and genetic backgrounds. Furthermore we investigated role of regulatory T cells (Tregs) in AIH and of polyspecific Tregs as therapeutic…