ABSTRACT

Mice with alterations in specific genes important for regulatory T (Treg) cell development, maintenance, and function develop spontaneous autoimmunity. The supreme manifestation is spontaneous systemic autoimmunity and occurs in the case of genes with critical roles in Treg cells, such as Foxp3. Several mouse models with genetic alterations causing spontaneous systemic autoimmunity are presented, some with germline deletions, others with conditional or inducible deletion in T cells or only in Treg cells. Additionally, the pathology, the immune populations, and the molecular mechanisms underlying the observed pathology in these mice are described, together with the various immunological techniques used to investigate the autoimmune pathogenesis within these mice.